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Surgery_Schwartz. prog-nosis, and pulmonary reserve. General indications for urgent surgery are presented in Table 19-22. In patients with significant cavitary disease or with fungus balls, the walls of the cavities are eroded and necrotic; rebleeding will likely ensue. In addi-tion, bleeding from cavitary lesions may be due to pulmonary artery erosion, which requires surgery for control.End-Stage Lung DiseaseLung Volume Reduction Surgery. The ideal patient for lung volume reduction surgery (LVRS) has heterogeneous emphy-sema with apical predominance, meaning the worst emphyse-matous changes are in the apex (seen on chest CT scan) of the lungs. The physiologic lack of function of these areas is dem-onstrated by quantitative perfusion scan, which shows minimal or no perfusion. By surgically excising these nonfunctional areas, the volume of the lung is reduced, theoretically restor-ing respiratory mechanics. Diaphragm position and function are improved, and there may be an improvement in the dynamic


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Surgery_Schwartz. nonfunctional areas, the volume of the lung is reduced, theoretically restor-ing respiratory mechanics. Diaphragm position and function are improved, and there may be an improvement in the dynamic small airway collapse in the remaining lung.Operative mortality in the initial experience was 16.9%, with a 1-year mortality of 23%. In response, the National Emphysema Treatment Trial (NETT) performed a randomized trial of 1218 patients in a noncrossover design to medical versus surgical management after a 10-week pretreatment pulmonary rehabilitation program. Subgroup analysis demonstrated that in patients with the anatomic changes delineated by Cooper and colleagues, LVRS significantly improved exercise capacity, lung function, quality of life, and dyspnea compared to medi-cal therapy. After 2 years, functional improvements began to decline toward baseline. Similar parameters in medically treated patients steadily decline below baseline. LVRS was associated with increased short-term


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Surgery_Schwartz. 2 years, functional improvements began to decline toward baseline. Similar parameters in medically treated patients steadily decline below baseline. LVRS was associated with increased short-term morbidity and mortality and did not confer a survival benefit over medical therapy.122Table 19-22General indications for urgent operative intervention for massive hemoptysis 1. Presence of a fungus ball 2. Presence of a lung abscess 3. Presence of significant cavitary disease 4. Failure to control the bleedingBrunicardi_Ch19_p0661-p0750.indd 71901/03/19 7:01 PM 720SPECIFIC CONSIDERATIONSPART IISurvival123BOS-free survival1.00.80.60.40.2Years posttransplantFigure 19-36. The survival rate after lung transplantation in the absence of bronchiolitis obliterans syndrome (BOS) at the University of Minnesota.Survival123Primary graft failure1.00.80.60.40.2No PGFPGFYears posttransplantFigure 19-37. The survival rate after lung transplantation at the University of Minnesota as a function of primary


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Surgery_Schwartz. graft failure1.00.80.60.40.2No PGFPGFYears posttransplantFigure 19-37. The survival rate after lung transplantation at the University of Minnesota as a function of primary graft failure (PGF).Lung Transplantation. The most common indications for lung transplant are COPD and idiopathic pulmonary fibrosis (IPF). Most patients with IPF and older patients with COPD are offered a single-lung transplant. Younger COPD patients and patients with α1-antitrypsin deficiency and severe hyperinflation of the native lungs are offered a bilateral-lung transplant. Most patients with primary pulmonary hypertension and almost all patients with cystic fibrosis are treated with a bilateral-lung transplant. A heart-lung transplant is reserved for patients with irreversible ventricular failure or uncorrectable congenital cardiac disease.Patients with COPD are considered for placement on the transplant waiting list when their FEV1 has fallen to below 25% of its predicted value. Patients with significant


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Surgery_Schwartz. congenital cardiac disease.Patients with COPD are considered for placement on the transplant waiting list when their FEV1 has fallen to below 25% of its predicted value. Patients with significant pulmonary hyper-tension should be listed earlier. IPF patients should be referred when their forced vital capacity has fallen to less than 60% or their Dlco has fallen to less than 50% of their predicted values.In the past, patients with primary pulmonary hypertension and New York Heart Association (NYHA) class III or IV symp-toms were listed for a lung transplant. However, treatment of such patients with intravenous prostacyclin and other pulmonary vasodilators has now markedly altered that strategy. Virtually all patients with primary pulmonary hypertension are now treated with intravenous epoprostenol. Several of these patients have experienced a marked improvement in their symptoms associ-ated with a decrease in their pulmonary arterial pressures and an increase in exercise capacity.


Surgery_Schwartz

Surgery_Schwartz. epoprostenol. Several of these patients have experienced a marked improvement in their symptoms associ-ated with a decrease in their pulmonary arterial pressures and an increase in exercise capacity. Listing of these patients is deferred until they develop NYHA class III or IV symptoms or until their mean pulmonary artery pressure rises above 75 mmHg.Medium-term and bronchiolitis obliterans syndrome (BOS)–free survival rates of patients who underwent a lung transplant at the University of Minnesota are shown in Figs. 19-35 and 19-36. The mortality of patients while waiting for transplants is about 10%. In an effort to expand the number of lung donors, many transplant groups have liberalized their criteria for donor selection. Still, the partial pressure of arterial oxygen (Pao2) should be greater than 300 mmHg on a fraction of inspired oxygen (Fio2) of 100%. In special circumstances, lungs may be used from donors with a smoking history; from donors older than 50 years of age; and from


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Surgery_Schwartz. greater than 300 mmHg on a fraction of inspired oxygen (Fio2) of 100%. In special circumstances, lungs may be used from donors with a smoking history; from donors older than 50 years of age; and from donors with positive Gram stains or infiltrates on CXR.123,124 The use of two living donors, each donating a single lower lobe, is another strategy for increasing the donor pool. Recipient outcomes are similar to those with cadaver donors in carefully selected patients.Survival123Overall survival1.00.80.60.40.2Years posttransplantFigure 19-35. The overall survival rate after lung transplantation at the University of Minnesota.Most of the early mortality after lung transplant is related to primary graft failure resulting from a severe ischemia-reper-fusion injury to the lung(s) (Fig. 19-37). Reperfusion injury is characterized radiographically by interstitial and alveolar edema and clinically by hypoxia and ventilation-perfusion mismatch. Donor neutrophils and recipient lymphocytes


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Surgery_Schwartz. Reperfusion injury is characterized radiographically by interstitial and alveolar edema and clinically by hypoxia and ventilation-perfusion mismatch. Donor neutrophils and recipient lymphocytes prob-ably play an important role in the pathogenesis of reperfu-sion injury. The most important impediment to longer-term survival after a lung transplant is the development of BOS, a manifestation of chronic rejection. Episodes of acute rejection are the major risk factors for developing BOS. Other injuries to the lung (including early reperfusion injury and chronic gas-troesophageal reflux disease) may also adversely affect long-term outcomes of patients.125,126CHEST WALLChest Wall MassClinical Approach. Surgeons confronted with a patient with a chest wall mass must be cognizant that their approach to diagno-sis and treatment has significant impact on the patient’s chances Brunicardi_Ch19_p0661-p0750.indd 72001/03/19 7:01 PM


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Surgery_Schwartz. CHAPTER 19721CHEST WALL, LUNG, MEDIASTINUM, AND PLEURADiagnosis is clear;a surgical resection is theprimary treatmentLesion <2.0 cmBenign Tumors Fibrous dysplasia Chondroma Osteochondroma Eosinophilic granulomaMalignant Tumors ChondrosarcomaWide surgical excisionCT or MRI or bothChest wall massFigure 19-38. Systematic approach for evaluating a chest wall mass when the clinical scenario is uncomplicated and initial imag-ing studies suggest a clear diagnosis. CT = computed tomography; MRI = magnetic resonance imaging.for long-term survival. All chest wall tumors should be consid-ered malignant until proven otherwise. It is critically important that the surgeon(s) be mindful of this tenet and well versed in the diagnostic and treatment principles for chest wall malignan-cies. These tenets must be applied from the initial biopsy, as the placement of the incision can impact significantly on the successful complete resection and reconstruction of the chest wall. Complete resection is


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Surgery_Schwartz. must be applied from the initial biopsy, as the placement of the incision can impact significantly on the successful complete resection and reconstruction of the chest wall. Complete resection is imperative if there is any hope for cure and/or long-term survival. A general approach is outlined in Figs. 19-38 and 19-39.Patients with chest wall tumors, regardless of etiology, typically complain of a slowly enlarging palpable mass (50% to 70%), chest wall pain (25% to 50%), or both. Interestingly, growing masses are often not noticed by the patient until they suffer a trauma to the area. Pain from a chest wall mass is typi-cally localized to the area of the tumor; it occurs more often and more intensely with malignant tumors, but it can also be present in up to one-third of patients with benign tumors. With Ewing’s sarcoma, fever and malaise may also be present. Benign chest wall tumors tend to occur in younger patients (average age 26 years), whereas malignant tumors tend to be found


Surgery_Schwartz

Surgery_Schwartz. tumors. With Ewing’s sarcoma, fever and malaise may also be present. Benign chest wall tumors tend to occur in younger patients (average age 26 years), whereas malignant tumors tend to be found in older patients (average age 40 years). Overall, between 50% and 80% of chest wall tumors are malignant.Evaluation and Management. Laboratory evaluations are useful in assessing chest wall masses for the following:1. Plasmacytoma. Serum protein electrophoresis demon-strates a single monoclonal spike, which is measuring the overproduction of one immunoglobulin from the malignant plasma cell clone.Chest wall massCT or MRI or bothDiagnosis is NOT clearPreoperative chemotherapyWide surgical excisionOsteosarcomaRhabdomyosarcomaNonrhabdomyosarcomaPNET/Ewing’s sarcomaNonrhabdosarcoma Fibrosarcoma Malignant fibrous histiocytoma Liposarcoma Synovial cell sarcoma DesmoidNeedle biopsyor incisional biopsyFigure 19-39. Systematic approach for evaluating a chest wall mass for which the diagnosis is


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Surgery_Schwartz. fibrous histiocytoma Liposarcoma Synovial cell sarcoma DesmoidNeedle biopsyor incisional biopsyFigure 19-39. Systematic approach for evaluating a chest wall mass for which the diagnosis is not unequivocal. A tissue diagnosis is critical for effective management of chest wall masses. CT = computed tomography; MRI = magnetic resonance imaging; PNET = primitive neuroectodermal tumor.2. Osteosarcoma. Alkaline phosphatase levels may be elevated.3. Ewing’s sarcoma. Erythrocyte sedimentation rates may be elevated.Radiography CXR may reveal rib destruction, calcification within the lesion, and if old films are available, a clue to growth rate. CT scanning, however, is necessary to determine the rela-tionship of the chest wall mass to contiguous structures (e.g., mediastinum, lung, soft tissues, and other skeletal elements), evaluate for pulmonary metastases, and assess for extraosseous bone formation and bone destruction, both typically seen with osteosarcoma.Because MRI provides


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Surgery_Schwartz. tissues, and other skeletal elements), evaluate for pulmonary metastases, and assess for extraosseous bone formation and bone destruction, both typically seen with osteosarcoma.Because MRI provides multiple planes of imaging (coronal, sagittal, and oblique), better definition of the relationship between tumor and muscle, and tumor and contiguous or nearby neurovascular structures or the spine, it is an important radio-graphic adjunct for preoperative planning. Compared to CT scan alone, MRI may further delineate tissue abnormalities, poten-tially enhancing the ability to distinguish benign from malignant sarcoma.Biopsy The first step in the management of all chest wall tumors is to obtain a tissue diagnosis. Inappropriate or mis-guided attempts at tissue diagnosis through casual open biopsy techniques have the potential (if the lesion is a sarcoma) to seed surrounding tissues and contiguous body cavities (e.g., the pleural space) with tumor cells, potentially compromising local


Surgery_Schwartz

Surgery_Schwartz. biopsy techniques have the potential (if the lesion is a sarcoma) to seed surrounding tissues and contiguous body cavities (e.g., the pleural space) with tumor cells, potentially compromising local Brunicardi_Ch19_p0661-p0750.indd 72101/03/19 7:01 PM 722SPECIFIC CONSIDERATIONSPART IItumor control and patient survival. Tissue diagnosis is accom-plished using one of three methods: needle biopsy (typically CT-guided, FNA, or a core biopsy), incisional biopsy, or exci-sional biopsy in limited and specific situations.1. Needle biopsy. Pathologists experienced with sarcomas can accurately diagnose approximately 90% of patients using FNA cytology. A needle biopsy (FNA or core) has the advantage of avoiding wound and body cavity contami-nation (a potential complication with an incisional biopsy).2. Incisional biopsy. If a needle biopsy is nondiagnostic, an incisional biopsy may be performed, with caveats. First, the skin incision must be placed directly over the mass and ori-ented to


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Surgery_Schwartz. biopsy).2. Incisional biopsy. If a needle biopsy is nondiagnostic, an incisional biopsy may be performed, with caveats. First, the skin incision must be placed directly over the mass and ori-ented to allow subsequent scar excision and skin flaps, and drains should be avoided. If the surgeon believes a hema-toma is likely to develop, a drain is useful for limiting soft tissue contamination by tumor cells. At the time of definitive surgical resection, the en bloc resection includes the biopsy scar and the drain tract along with the tumor.3. Excisional biopsy. Any lesion less than 2.0 cm can be excised as long as the resulting wound is small enough to close primarily. Otherwise, excisional biopsy is per-formed only when the initial diagnosis (based on radio-graphic evaluation) indicates that the lesion is benign or when the lesion has the classic appearance of a chondro-sarcoma (in which case, definitive surgical resection can be undertaken).Benign Chest Wall Neoplasms1. Chondroma.


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Surgery_Schwartz. that the lesion is benign or when the lesion has the classic appearance of a chondro-sarcoma (in which case, definitive surgical resection can be undertaken).Benign Chest Wall Neoplasms1. Chondroma. Chondromas, seen primarily in children and young adults, are one of the more common benign tumors of the chest wall. They usually occur at the costochondral junction anteriorly and may be confused with costochondri-tis, except that a painless mass is present. Radiographically, the lesion is lobulated and radiodense; it may have diffuse or focal calcifications; and it may displace the bony cortex without penetration. Chondromas may grow to huge sizes if left untreated. Treatment is surgical resection with a 2-cm margin. Large chondromas may harbor well-differentiated chondrosarcoma and should be managed with a 4-cm mar-gin to prevent local recurrence.1272. Fibrous dysplasia. As with chondromas, fibrous dysplasia most frequently occurs in young adults and may be associ-ated with trauma. Pain


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Surgery_Schwartz. managed with a 4-cm mar-gin to prevent local recurrence.1272. Fibrous dysplasia. As with chondromas, fibrous dysplasia most frequently occurs in young adults and may be associ-ated with trauma. Pain is an infrequent complaint, and the lesion is typically located in the posterolateral aspect of the rib cage. Radiographically, an expansile mass is present, with cortical thinning and no calcification. Local excision with a 2-cm margin is curative.3. Osteochondroma. Osteochondromas, often found inciden-tally as a solitary lesion on radiograph, are the most common benign bone tumor. Osteochondromas occur in the first two decades of life, and they arise at or near the growth plate of bones. Osteochondromas in the thorax arise from the rib cortex. They are one of several components to the autosomal dominant syndrome, hereditary multiple exostoses. When part of this syndrome, osteochondromas have a high rate of degeneration into chondrosarcomas. Any patient with heredi-tary multiple exostoses


Surgery_Schwartz

Surgery_Schwartz. dominant syndrome, hereditary multiple exostoses. When part of this syndrome, osteochondromas have a high rate of degeneration into chondrosarcomas. Any patient with heredi-tary multiple exostoses syndrome who develops new pain at the site of an osteochondroma or who notes gradual growth in the mass over time should be carefully evaluated for osteosarcoma. Local excision of a benign osteochondroma is sufficient. If malignancy is determined, wide excision is performed with a 4-cm margin.4. Eosinophilic granuloma. Eosinophilic granulomas are benign osteolytic lesions. Eosinophilic granulomas of the ribs can occur as solitary lesions or as part of a more gener-alized disease process of the lymphoreticular system termed Langerhans cell histiocytosis (LCH). In LCH, the involved tissue is infiltrated with large numbers of histiocytes (similar to Langerhans cells seen in skin and other epithelia), which are often organized as granulomas. The cause is unknown. Of all LCH bone lesions, 79% are


Surgery_Schwartz

Surgery_Schwartz. with large numbers of histiocytes (similar to Langerhans cells seen in skin and other epithelia), which are often organized as granulomas. The cause is unknown. Of all LCH bone lesions, 79% are solitary eosinophilic granulomas, 7% involve multiple eosinophilic granulomas, and 14% belong to other forms of more systemic LCH. Iso-lated single eosinophilic granulomas can occur in the ribs or skull, pelvis, mandible, humerus, and other sites. They are diagnosed primarily in children between the ages of 5 and 15 years. Because of the associated pain and tenderness, they may be confused with Ewing’s sarcoma or with an inflammatory process such as osteomyelitis. Healing may occur spontaneously, but the typical treatment is limited sur-gical resection with a 2-cm margin.5. Desmoid tumors. Soft tissue neoplasms arising from fas-cial or musculoaponeurotic structures, desmoid tumors con-sist of proliferations of benign-appearing fibroblastic cells, abundant collagen, and few mitoses. Desmoid


Surgery_Schwartz

Surgery_Schwartz. tissue neoplasms arising from fas-cial or musculoaponeurotic structures, desmoid tumors con-sist of proliferations of benign-appearing fibroblastic cells, abundant collagen, and few mitoses. Desmoid tumors pos-sess alterations in the adenomatous polyposis coli (APC)/β-catenin pathway. Cyclin D1 dysregulation is thought to play a significant role in their pathogenesis.128 Associations with other diseases and conditions are well documented, espe-cially those with similar alterations in the APC pathway, such as familial adenomatous polyposis (Gardner’s syn-drome). Other conditions with increased risk of desmoid tumor formation include increased estrogen states (preg-nancy) and trauma. Surgical incisions (abdominal and tho-rax) have been the site of desmoid development, either in or near the scar. Clinically, patients are usually in the third to fourth decade of life and have pain, a chest wall mass, or both. The tumor is usually fixed to the chest wall, but not to the over-lying skin.


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Surgery_Schwartz. scar. Clinically, patients are usually in the third to fourth decade of life and have pain, a chest wall mass, or both. The tumor is usually fixed to the chest wall, but not to the over-lying skin. There are no typical radiographic findings, but MRI may delineate muscle or soft tissue infiltration. Des-moid tumors do not metastasize, but they have a significant propensity to recur locally, with rates as high as 5% to 50%, sometimes despite complete initial resection with histologi-cally negative margins.129 Such locally aggressive behavior is secondary to microscopic tumor infiltration of muscle and surrounding soft tissues and prompts some to consider them a low-grade form of fibrosarcoma. Because the lesions have low cellularity and poor yield with FNA, an open incisional biopsy for lesions over 3 to 4 cm is often necessary, following the caveats listed ear-lier (see biopsy section). Surgery consists of wide local excision with a 2to 4-cm margin and intraoperative fro-zen section


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Surgery_Schwartz. over 3 to 4 cm is often necessary, following the caveats listed ear-lier (see biopsy section). Surgery consists of wide local excision with a 2to 4-cm margin and intraoperative fro-zen section assessment of resection margins. Typically, chest wall resection, including the involved rib(s) and one rib above and below the tumor with a 4to 5-cm margin of rib, is required. A margin of less than 1 cm results in much higher local recurrence rates. If a major neurovascu-lar structure would have to be sacrificed, leading to high morbidity, then a margin of less than 1 cm would have to suffice. Survival after wide local excision with negative margins is 90% at 10 years.130Brunicardi_Ch19_p0661-p0750.indd 72201/03/19 7:01 PM


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Surgery_Schwartz. CHAPTER 19723CHEST WALL, LUNG, MEDIASTINUM, AND PLEURAPrimary Malignant Chest Wall TumorsMalignant tumors of the chest wall are either metastatic lesions from another primary tumor or sarcoma. Soft tissue sarcomas of the chest wall include fibrosarcomas, liposarcomas, malignant fibrous histiocytomas (MFHs), rhabdomyosarcomas, angiosar-comas, and other extremely rare lesions (Fig. 19-40). Despite the prevalence of localized disease, soft tissue sarcomas of the chest wall have significantly worse survival than similar tumors located on the extremities or the head and neck region. The fac-tors impacting on risk of death from soft tissue sarcomas of the chest wall are presented in Table 19-23. All sarcomas have a propensity to spread to the lungs.While many varieties of sarcoma exist, the primary features affecting prognosis are histologic grade and respon-siveness to chemotherapy (Table 19-24). Preoperative (neo-adjuvant) chemotherapy offers the ability to (a) assess tumor


Surgery_Schwartz

Surgery_Schwartz. exist, the primary features affecting prognosis are histologic grade and respon-siveness to chemotherapy (Table 19-24). Preoperative (neo-adjuvant) chemotherapy offers the ability to (a) assess tumor chemosensitivity by the degree of tumor size reduction and microscopic necrosis; (b) determine tumor sensitivity to spe-cific chemotherapeutic agents; and (c) improve resectability by reducing tumor size. Patients whose tumors are responsive to preoperative chemotherapy have a much better prognosis than those with a poor response. Information about tumor response to chemotherapy, the patient’s physiologic state and capacity to receive treatment, and metastatic disease status is used to determine optimal therapy. The initial treatment is either (a) preoperative chemotherapy (for patients with osteosarcoma, rhabdomyosarcoma, primitive neuroectoder-mal tumor, or Ewing’s sarcoma) followed by surgery and postoperative chemotherapy; (b) primary surgical resection and reconstruction (for


Surgery_Schwartz

Surgery_Schwartz. with osteosarcoma, rhabdomyosarcoma, primitive neuroectoder-mal tumor, or Ewing’s sarcoma) followed by surgery and postoperative chemotherapy; (b) primary surgical resection and reconstruction (for patients with nonmetastatic MFH, fibrosarcoma, liposarcoma, or synovial sarcoma); or (c) preoperative chemotherapy followed by surgical resection if indicated in patients presenting with metastatic soft tis-sue sarcomas. Contiguous involvement of underlying lung or other soft tissues or the presence of pulmonary metasta-ses does not preclude successful surgery. In fact, patients receiving surgical intervention have significantly better overall survival. Median survival with surgical resection is 25 months compared to 8 months without resection. Addi-tional prognostic variables that are important for long-term survival include tumor size, grade, stage, and negative re-resection margin.131 With the exception of rhabdomyosar-comas, the primary treatment of these lesions is wide surgical


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Surgery_Schwartz. for long-term survival include tumor size, grade, stage, and negative re-resection margin.131 With the exception of rhabdomyosar-comas, the primary treatment of these lesions is wide surgical resection with 4-cm margins and reconstruction.132The following is an overview of several chest wall sarcomas.1. Chondrosarcoma. Chondrosarcomas are the most common primary chest wall malignancy. As with chondromas, they usually arise anteriorly from the costochondral arches. CT scan shows a radiolucent lesion often with stippled calcifica-tions pathognomonic for chondrosarcomas (Fig. 19-41). The involved bony structures are also destroyed. Most chondro-sarcomas are slow-growing, low-grade tumors; these often painful masses can reach massive proportions.127 For this reason, any lesion in the anterior chest wall likely to be a low-grade chondrosarcoma should be treated with wide (4-cm) resection after metastatic disease to the lungs or bones is ruled out. Chondrosarcomas are not sensitive to


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Surgery_Schwartz. chest wall likely to be a low-grade chondrosarcoma should be treated with wide (4-cm) resection after metastatic disease to the lungs or bones is ruled out. Chondrosarcomas are not sensitive to radiation or chemo-therapy. Prognosis is determined by tumor grade and extent of resection. With a low-grade tumor and wide resection, patient survival at 5 to 10 years can be as high as 60% to 80%.2. Osteosarcoma. While osteosarcomas are the most com-mon bone malignancy, they represent only 10% to 15% of all malignant chest wall tumors.133,134 They primarily occur in young adults as rapidly enlarging, painful masses; how-ever, osteosarcomas can occur in older patients as well, Figure 19-40. Chest computed tomography scan showing a right chest wall tumor (arrow). Tissue diagnosis revealed that this mass was a leiomyosarcoma.Brunicardi_Ch19_p0661-p0750.indd 72301/03/19 7:01 PM 724SPECIFIC CONSIDERATIONSPART IITable 19-23Cox proportional hazards model for risk of death from soft tissue


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Surgery_Schwartz. this mass was a leiomyosarcoma.Brunicardi_Ch19_p0661-p0750.indd 72301/03/19 7:01 PM 724SPECIFIC CONSIDERATIONSPART IITable 19-23Cox proportional hazards model for risk of death from soft tissue sarcomaNHAZARD RATIO95% CIP VALUEGender Male Female39374113Reference group0.897Reference group0.843–0.955Reference group.001Age 50 years 51–70 years >70 years183730993114Reference group1.1311.538Reference group1.026–1.2471.395–1.697Reference group.013<.001Race Caucasian Non-Caucasian7152898Reference group1.212Reference group1.093–1.344Reference group<.001Histologic type Fibrosarcoma MFH Liposarcoma LMS/GIST489252915343498Reference group1.2810.8941.204Reference group1.097–1.4950.759–1.0541.033–1.403Reference group.002.182.018Location Head and neck Trunk Extremity Retroperitoneum57640542474946Reference group1.2551.0031.276Reference group1.096–1.4380.875–1.1511.093–1.489Reference group.001.960.002Stage Localized Regional Distant500617241320Reference group1.5752.897Reference


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Surgery_Schwartz. group1.2551.0031.276Reference group1.096–1.4380.875–1.1511.093–1.489Reference group.001.960.002Stage Localized Regional Distant500617241320Reference group1.5752.897Reference group1.458–1.7022.660–3.155Reference group<.001<.001Surgical treatment Yes No67541296Reference group1.562Reference group1.443–1.691Reference group<.001Radiation therapy Yes No21755875Reference group1.151Reference group1.070–1.239Reference group<.001Chemotherapy Yes No10626988Reference group0.909Reference group0.829–0.996Reference group.041Abbreviations: CI = confidence interval; GIST = gastrointestinal stromal tumor; LMS = leiomyosarcoma; MFH = malignant fibrous histiocytoma.Reproduced with permission from Gutierrez JC, Perez EA, Franceschi D, et al: Outcomes for soft-tissue sarcoma in 8249 cases from a large state cancer registry, J Surg Res. 2007;141(1):105-114.Table 19-24Classification of sarcomas by therapeutic responseTUMOR TYPECHEMOTHERAPY SENSITIVITYOsteosarcoma+Rhabdomyosarcoma+Primitive neuroectodermal


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Surgery_Schwartz. registry, J Surg Res. 2007;141(1):105-114.Table 19-24Classification of sarcomas by therapeutic responseTUMOR TYPECHEMOTHERAPY SENSITIVITYOsteosarcoma+Rhabdomyosarcoma+Primitive neuroectodermal tumor+Ewing’s sarcoma+Malignant fibrous histiocytoma±Fibrosarcoma±Liposarcoma±Synovial sarcoma±sometimes in association with previous radiation, Paget’s disease, or chemotherapy. Radiographically, the typical appearance consists of spicules of new periosteal bone formation producing a sunburst appearance. Osteosarcomas have a propensity to spread to the lungs, and up to one-third of patients present with metastatic disease. Osteosarcomas are potentially sensitive to chemotherapy. Currently, pre-operative chemotherapy is common. After chemotherapy, complete resection is performed with wide (4-cm) margins, followed by reconstruction. In patients presenting with lung metastases that are potentially amenable to surgical resection, induction chemotherapy may be given, followed by surgical resection


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Surgery_Schwartz. followed by reconstruction. In patients presenting with lung metastases that are potentially amenable to surgical resection, induction chemotherapy may be given, followed by surgical resection of the primary tumor and of the pul-monary metastases. Following surgical treatment of known disease, additional maintenance chemotherapy is usually recommended.Brunicardi_Ch19_p0661-p0750.indd 72401/03/19 7:01 PM


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Surgery_Schwartz. CHAPTER 19725CHEST WALL, LUNG, MEDIASTINUM, AND PLEURAFigure 19-41. Chest computed tomography scan showing a right posterior lung tumor. In the appropriate clinical setting, stippled calcifica-tions (white streaks in right lung mass) are highly indicative of chondrosarcomas.3. Malignant fibrous histiocytoma. Originally thought to derive from histiocytes because of the microscopic appear-ance of cultured tumor cells, these tumors likely originate from the fibroblast. MFHs are generally the most common soft tissue sarcoma of late adult life, although they are rare on the chest wall. The typical age at presentation is between age 50 and 70 years. Presentation is pain, with or without a palpable mass. Radiographically, a mass is usually evident, with destruction of surrounding tissue and bone. Treatment is wide resection with a margin of 4 cm or more and recon-struction. Over two-thirds of patients suffer from distant metastasis or local recurrence.4. Liposarcoma. Liposarcomas make up 15%


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Surgery_Schwartz. Treatment is wide resection with a margin of 4 cm or more and recon-struction. Over two-thirds of patients suffer from distant metastasis or local recurrence.4. Liposarcoma. Liposarcomas make up 15% of chest wall sarcomas. Most liposarcomas are low-grade tumors that have a propensity to recur locally, given their infiltrative nature. They typically present as a painless mass. Treatment is wide resection and reconstruction. Intraoperative mar-gins should be evaluated (as with all sarcomas) and resec-tion continued, if feasible, until margins are negative. Local recurrence can be treated with reexcision, with occasional use of radiotherapy.5. Fibrosarcoma. Often presenting as a large, painful mass, these lesions are visible on plain radiograph or CT, with sur-rounding tissue destruction. Treatment is wide local excision with intraoperative frozen-section analysis of margins, fol-lowed by reconstruction. Local and systemic recurrence is frequent. Patient survival at 5 years is about 50%


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Surgery_Schwartz. is wide local excision with intraoperative frozen-section analysis of margins, fol-lowed by reconstruction. Local and systemic recurrence is frequent. Patient survival at 5 years is about 50% to 60%.6. Rhabdomyosarcoma. Rhabdomyosarcomas are rare tumors of the chest wall. Microscopically, they are a spindle cell tumor. The diagnosis often depends on immunohistochemi-cal staining for muscle markers. Rhabdomyosarcomas are sensitive to chemotherapy. Treatment consists of preopera-tive chemotherapy with subsequent surgical resection.Other Tumors of the Chest Wall1. Primitive neuroectodermal tumors (PNETs) and Ewing’s sarcoma. PNETs (neuroblastomas, ganglioneuroblastomas, and ganglioneuromas) derive from primordial neural crest cells that migrate from the mantle layer of the developing spinal cord. Histologically, PNETs and Ewing’s sarcomas are small, round cell tumors; both possess a translocation between the long arms of chromosomes 11 and 22 within their genetic makeup. They also share


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Surgery_Schwartz. cord. Histologically, PNETs and Ewing’s sarcomas are small, round cell tumors; both possess a translocation between the long arms of chromosomes 11 and 22 within their genetic makeup. They also share a consistent pattern of proto-oncogene expression and have been found to express the product of the MIC2 gene. Ewing’s sarcoma occurs in adolescents and young adults who present with progressive chest wall pain, but without the presence of a mass. Systemic symptoms of malaise and fever are often present. Laboratory studies reveal an ele-vated erythrocyte sedimentation rate and mild white blood cell elevation. Radiographically, the characteristic onion peel appearance is produced by multiple layers of perios-teum in the bone formation. Evidence of bony destruction is also common. The diagnosis can be made by a percutaneous needle biopsy or an incisional biopsy. These tumors have a strong propensity to metastasize to the lungs and skeleton; patient survival rates are thus only 50% or less


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Surgery_Schwartz. can be made by a percutaneous needle biopsy or an incisional biopsy. These tumors have a strong propensity to metastasize to the lungs and skeleton; patient survival rates are thus only 50% or less at 3 years. Increasing tumor size is associated with decreasing survival. Treatment has improved sig-nificantly and now consists of multiagent chemotherapy, radiation therapy, and surgery. Patients are typically treated preoperatively with chemotherapy and reevaluated with radiologic imaging. When residual disease is identified, sur-gical resection and reconstruction are performed followed by maintenance chemotherapy.2. Plasmacytoma. Solitary plasmacytomas of the chest wall are very rare, with approximately 25 to 30 cases per year in the United States.133 The typical presentation is pain with-out a palpable mass. Plain radiographs show an osteolytic lesion in the region of the pain. As with other chest wall tumors, a needle biopsy under CT guidance is performed for diagnosis.


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Surgery_Schwartz. is pain with-out a palpable mass. Plain radiographs show an osteolytic lesion in the region of the pain. As with other chest wall tumors, a needle biopsy under CT guidance is performed for diagnosis. Histologically, the lesion is identical to multiple myeloma, with sheets of plasma cells. It occurs at an aver-age age of 55 years. Evaluation for systemic myeloma is performed with bone marrow aspiration, testing of calcium levels, and measurement of urinary Bence Jones proteins. If the results of these studies are negative, then a solitary Brunicardi_Ch19_p0661-p0750.indd 72501/03/19 7:01 PM 726SPECIFIC CONSIDERATIONSPART IIplasmacytoma is diagnosed. Surgery is usually limited to a biopsy only, which may be excisional.134 Treatment consists of radiation with doses of 4000 to 5000 cGy. Up to 75% of patients develop systemic multiple myeloma with 10-year survival of approximately 20%.Chest Wall ReconstructionThe primary determinant of long-term freedom from recurrence and overall


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Surgery_Schwartz. Up to 75% of patients develop systemic multiple myeloma with 10-year survival of approximately 20%.Chest Wall ReconstructionThe primary determinant of long-term freedom from recurrence and overall survival is margin status; therefore, adequate mar-gins of normal tissue must be included in the en bloc resec-tion. En bloc resection should include involved ribs, sternum, superior sulcus, or spine if necessary; invasion of these struc-tures should not be considered a contraindication to surgery in an otherwise fit patient. The resection should include at least one normal adjacent rib above and below the tumor, with all intervening intercostal muscles and pleura. In addition, an en bloc resection of overlying chest wall muscles is often neces-sary, such as of the pectoralis minor or major, serratus anterior, or latissimus dorsi. When the periphery of the lung is involved with the neoplasm, it is appropriate to resect the adjacent part of the pulmonary lobe in continuity (Fig. 19-42).


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Surgery_Schwartz. serratus anterior, or latissimus dorsi. When the periphery of the lung is involved with the neoplasm, it is appropriate to resect the adjacent part of the pulmonary lobe in continuity (Fig. 19-42). Involvement of the sternum by a malignant tumor requires total resection of the sternum with the adjacent cartilage. Techniques for postop-erative respiratory support are now good enough that resection should not be compromised because of any concern about the patient’s ability to be adequately ventilated in the early postop-erative period.The extent of resection depends on the tumor’s location and on any involvement of contiguous structures. Laterally based lesions often require simple wide excision, with resec-tion of any contiguously involved lung, pleura, muscle, or skin. Anteriorly based lesions contiguous with the sternum require partial sternectomy. Primary malignant tumors of the sternum may require complete sternectomy. Posterior lesions involving the rib heads over their


Surgery_Schwartz

Surgery_Schwartz. based lesions contiguous with the sternum require partial sternectomy. Primary malignant tumors of the sternum may require complete sternectomy. Posterior lesions involving the rib heads over their articulations with the vertebral bodies may, depending on the extent of rib involvement, require partial en bloc vertebrectomy.Optimal management of larger tumors includes care-ful preoperative planning and execution of the surgery by the thoracic surgeon and an experienced plastic surgeon in order to ensure optimal physiologic and cosmetic results. With these measures, reconstruction at the same operation can be accomplished.135 Reconstruction of a large defect in the chest wall requires the use of some type of material to prevent lung herniation and to provide stability for the chest wall (see Fig. 19-42). Mild degrees of paradoxical motion are often well tolerated if the area of instability is relatively small. Historically, a wide variety of materials have been used to reestablish chest


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Surgery_Schwartz. Fig. 19-42). Mild degrees of paradoxical motion are often well tolerated if the area of instability is relatively small. Historically, a wide variety of materials have been used to reestablish chest wall stability, including rib autografts, steel struts, acrylic plates, and numerous synthetic meshes. The current preference is either a 2-mm polytetrafluoroethylene (Gore-Tex) patch or a double-layer polypropylene (Marlex) mesh sandwiched with methylmethacrylate. There are sev-eral properties that make Gore-Tex an excellent material for use in chest wall reconstruction: (a) it is impervious to fluid, which prevents pleural fluid from entering the chest wall and minimizes the formation of seromas, which can compromise the myocutaneous flap viability and provide a nidus for infec-tion; and (b) it provides excellent rigidity and stability when secured taut to the surrounding bony structure and, as a result, provides a firm platform for myocutaneous flap reconstruc-tion. Except for smaller


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Surgery_Schwartz. it provides excellent rigidity and stability when secured taut to the surrounding bony structure and, as a result, provides a firm platform for myocutaneous flap reconstruc-tion. Except for smaller lesions, tissue coverage requires the use of myocutaneous flaps (latissimus dorsi, serratus anterior, rectus abdominis, or pectoralis major muscles).136,137MEDIASTINUMAnatomy and Pathologic EntitiesThe mediastinum can be divided into compartments for classi-fication of anatomic components and disease processes, which, despite substantial overlap, facilitates understanding of general concepts of surgical interest. Several classification schemes exist, but for the purposes of this chapter, the three-compart-ment model is used (Fig. 19-43). The anterior compartment lies between the sternum and the anterior surface of the heart and great vessels. The visceral or middle compartment is located between the great vessels and the trachea. As the name implies, the posterior compartment lies posterior


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Surgery_Schwartz. anterior surface of the heart and great vessels. The visceral or middle compartment is located between the great vessels and the trachea. As the name implies, the posterior compartment lies posterior and includes the para-vertebral sulci, bilaterally, and the paraesophageal area.ABFigure 19-42. Principles of recon-struction after resection of a chest wall tumor (osteogenic sarcoma) are shown. A. En bloc resection of the involved chest wall, including nor-mal ribs above and below the tumor as well as pulmonary parenchyma, must be performed. The resected specimen is shown. B. A prosthesis has been sewn in place. In the lower third of the prosthesis, the line of diaphragm reattachment is seen. The skin defect was closed with a myo-cutaneous flap from the ipsilateral rectus muscle.Brunicardi_Ch19_p0661-p0750.indd 72601/03/19 7:01 PM


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Surgery_Schwartz. CHAPTER 19727CHEST WALL, LUNG, MEDIASTINUM, AND PLEURAPosteriormediastinumAnterosuperiormediastinumMiddlemediastinumFigure 19-43. Anatomic division of the mediastinum.ThymusFigure 19-44. Normal appearance of the thymus gland in childhood. Ao = aorta; PA = pulmonary artery; VC = vena cava.The normal content of the anterior compartment includes the thymus gland or its remnant, the internal mammary artery and vein, lymph nodes, and fat. The thymus gland is large during childhood, occupying the entire anterior mediastinum (Fig. 19-44) but decreases in both thickness and length after adolescence and takes on a more fatty content, with only resid-ual islands of thymic cellular components (Fig. 19-45). The middle mediastinal compartment contains the pericardium and its contents, the ascending and transverse aorta, the superior and inferior venae cavae, the brachiocephalic artery and vein, the phrenic and upper vagus nerves, the trachea and main bronchi and corresponding lymph nodes, and the


Surgery_Schwartz

Surgery_Schwartz. transverse aorta, the superior and inferior venae cavae, the brachiocephalic artery and vein, the phrenic and upper vagus nerves, the trachea and main bronchi and corresponding lymph nodes, and the central portions of the pulmonary arteries and veins. The posterior compartment contains the descending aorta, esophagus, thoracic duct, azygos and hemiazygos veins, and lymph nodes. Numerous pathologic variants may be present in the various compartments, with much overlap. Table 19-25 includes the most common pathologic entities listed by compartment.138,139History and Physical ExaminationMediastinal pathology varies significantly by patient age. In children, neurogenic tumors of the posterior mediastinum are most common, followed by lymphoma, which is usually located in the anterior or middle compartment. Thymoma in child-hood is rare (Table 19-26). In adults, the most common tumors include neurogenic tumors of the posterior compartment, benign cysts occurring in any compartment, and


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Surgery_Schwartz. compartment. Thymoma in child-hood is rare (Table 19-26). In adults, the most common tumors include neurogenic tumors of the posterior compartment, benign cysts occurring in any compartment, and thymomas of the ante-rior mediastinum (Table 19-27). In both age groups, about 25% of mediastinal tumors are malignant. Pediatric tumors will be discussed in Chapter 39.Up to two-thirds of mediastinal tumors in adults are dis-covered as asymptomatic abnormalities on radiologic studies ordered for other problems, particularly now that screening CT examinations are more prevalent. When symptomatic, these tumors are significantly more likely to be malignant. Charac-teristics such as size, location, rate of growth, and associated inflammation are important factors that correlate with symp-toms. Large, bulky tumors, expanding cysts, and teratomas can cause compression of mediastinal structures, in particular the trachea, and lead to cough, dyspnea on exertion, or stridor. Chest pain or dyspnea may


Surgery_Schwartz

Surgery_Schwartz. bulky tumors, expanding cysts, and teratomas can cause compression of mediastinal structures, in particular the trachea, and lead to cough, dyspnea on exertion, or stridor. Chest pain or dyspnea may be reported secondary to associated pleural effusions, cardiac tamponade, or phrenic nerve involve-ment. Occasionally, a mediastinal mass near the aortopulmonary window may be identified in a workup for hoarseness because of left recurrent laryngeal nerve involvement. The patient in Fig. 19-46 presented with hoarseness due to nodal compression of the left recurrent laryngeal nerve from a primary lung cancer with metastases to the level 5 and 6 lymph nodes in the region of the aortopulmonary window.The history and physical examination in conjunction with the imaging findings may suggest a specific diagnosis (Table 19-28). In one series, systemic symptoms were present in 50% of patients with a mediastinal mass and a lymphoproliferative dis-order, as compared with only 29% of patients with


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Surgery_Schwartz. diagnosis (Table 19-28). In one series, systemic symptoms were present in 50% of patients with a mediastinal mass and a lymphoproliferative dis-order, as compared with only 29% of patients with other masses (such as thymic or neurogenic). Laboratory signs of inflamma-tion were also noted; the erythrocyte sedimentation rate and C-reactive protein levels were elevated and leukocytosis was present in 86% of patients with a lymphoproliferative disorder, as compared with only 58% of patients with other types of medi-astinal masses.Imaging and Serum MarkersChest CT or MRI is required to fully delineate the anatomy.140 A contrast-enhanced CT scan enables clear delineation of the Brunicardi_Ch19_p0661-p0750.indd 72701/03/19 7:01 PM 728SPECIFIC CONSIDERATIONSPART IITable 19-25Usual location of the common primary tumors and cysts of the mediastinumANTERIOR COMPARTMENTVISCERAL COMPARTMENTPARAVERTEBRAL SULCIThymomaEnterogenous cystNeurilemoma-schwannomaGerm cell


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Surgery_Schwartz. 19-25Usual location of the common primary tumors and cysts of the mediastinumANTERIOR COMPARTMENTVISCERAL COMPARTMENTPARAVERTEBRAL SULCIThymomaEnterogenous cystNeurilemoma-schwannomaGerm cell tumorLymphomaNeurofibromaLymphomaPleuropericardial cystMalignant schwannomaLymphangiomaMediastinal granulomaGanglioneuromaHemangiomaLymphoid hamartomaGanglioneuroblastomaLipomaMesothelial cystNeuroblastomaFibromaNeuroenteric cystParagangliomaFibrosarcomaParagangliomaPheochromocytomaThymic cystPheochromocytomaFibrosarcomaParathyroid adenomaThoracic duct cystLymphomaReproduced with permission from Shields TW: Mediastinal Surgery. Philadelphia, PA: Lea & Febiger; 1991.Table 19-26Mediastinal tumors in childrenTUMOR TYPEPERCENTAGE OF TOTALLOCATIONNeurogenic tumors40PosteriorLymphomas18Anterior/middleCysts18AllGerm cell tumors11AnteriorMesenchymal tumors9AllThymomasRareAnteriorReproduced with permission from Silverman NA, Sabiston DC: Mediastinal masses, Surg Clin North Am. 1980


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Surgery_Schwartz. cell tumors11AnteriorMesenchymal tumors9AllThymomasRareAnteriorReproduced with permission from Silverman NA, Sabiston DC: Mediastinal masses, Surg Clin North Am. 1980 Aug;60(4):757-777.Figure 19-45. Computed tomography scan showing the normal appearance of an involuted thymus gland in an adult. Note the near-total fatty appearance of the gland with only tiny islands of soft tissue scattered within it (small arrows).soft tissue structures from the vasculature and is preferred over noncontrast studies. If there is concern for invasion of vas-cular structures or spinal involvement, MRI is more accurate than CT scan and provides important information regarding respectability.If an endocrine origin is suspected, several other imaging modalities are available (Table 19-29). Single-photon emission CT (SPECT) technology may be used to improve image contrast and give information on three-dimensional localization, largely replacing conventional two-dimensional nuclear imaging studies. If a


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Surgery_Schwartz. CT (SPECT) technology may be used to improve image contrast and give information on three-dimensional localization, largely replacing conventional two-dimensional nuclear imaging studies. If a thyroid origin is suspected, a thyroid scan using 131I or 123I can identify most intrathoracic goiters and identify the extent of functioning thyroid tissue. If indicated, the thyroid scan should precede other scans requiring iodine-containing contrast agents because they would subsequently interfere with iodine tracer uptake by thyroid tissue. If a pheochromo-cytoma or neuroblastoma is suspected, the octreotide scan or 123I-metaiodobenzylguanidine (MIBG) scans are helpful in diag-nosis and localization. The sestamibi scan may be useful for diagnosing and localizing a mediastinal parathyroid gland. PET is useful for distinguishing malignant from benign tumors and may help detect distant metastases in some patients. However, the role of routine PET imaging for staging surgically resectable


Surgery_Schwartz

Surgery_Schwartz. PET is useful for distinguishing malignant from benign tumors and may help detect distant metastases in some patients. However, the role of routine PET imaging for staging surgically resectable lesions of the mediastinum has not been established.The use of serum markers to evaluate a mediastinal mass can be invaluable in some patients. For example, nonsemino-matous and seminomatous germ cell tumors can frequently be diagnosed and often distinguished from one another by the lev-els of α-fetoprotein (AFP) and human chorionic gonadotropin (hCG). In over 90% of nonseminomatous germ cell tumors, either the AFP or the hCG level will be elevated. Results are close to 100% specific if the level of either AFP or hCG is greater than 500 ng/mL. Some centers institute chemotherapy based on this result alone, without biopsy confirmation of the diagnosis. In contrast, the AFP level in patients with mediastinal seminoma is always normal; only 10% will have elevated hCG, which is usually less than


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Surgery_Schwartz. alone, without biopsy confirmation of the diagnosis. In contrast, the AFP level in patients with mediastinal seminoma is always normal; only 10% will have elevated hCG, which is usually less than 100 ng/mL. Other serum markers, such as intact parathyroid hormone level for ectopic parathyroid Brunicardi_Ch19_p0661-p0750.indd 72801/03/19 7:01 PM


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Surgery_Schwartz. CHAPTER 19729CHEST WALL, LUNG, MEDIASTINUM, AND PLEURAFigure 19-46. Computed tomography scan of a patient who pre-sented with hoarseness due to compression of the left recurrent laryngeal nerve caused by mediastinal lymph node metastases to the aortopulmonary window area (arrow) from a primary lung cancer.adenomas, may be useful for diagnosing and also for intraopera-tively confirming complete resection. After successful resection of a parathyroid adenoma, this hormone level should rapidly normalize.Diagnostic Nonsurgical Biopsies of the MediastinumThe treatment of up to 60% of patients with anterior mediastinal masses is ultimately nonsurgical, so it is essential to understand all options for obtaining adequate tissue for a definitive diagno-sis using the least invasive approach. CT-guided needle biopsy, EBUSand EUS-guided FNA, and even core-needle biopsy (either CT-guided and EUS-guided) have proven most useful for cytologic and tissue diagnosis of mediastinal masses and


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Surgery_Schwartz. CT-guided needle biopsy, EBUSand EUS-guided FNA, and even core-needle biopsy (either CT-guided and EUS-guided) have proven most useful for cytologic and tissue diagnosis of mediastinal masses and lymphadenopathy.When FNA and core-needle biopsy were combined, the accuracy was 98%, compared to 79% for each modality inde-pendently. In addition, core-needle biopsy changed the diagno-sis in nine cases that had been missed by FNA due to inadequate specimens. Finally, core-needle biopsy was better at diagnosis for benign diseases compared to FNA. Accessible nodal stations Table 19-27Mediastinal tumors in adultsTUMOR TYPEPERCENTAGE OF TOTALLOCATIONNeurogenic tumors21PosteriorCysts20AllThymomas19AnteriorLymphomas13Anterior/middleGerm cell tumors11AnteriorMesenchymal tumors7AllEndocrine tumors6Anterior/middleData from Shields TW: General Thoracic Surgery, 4th ed. Baltimore, MD: Lippincott Williams & Wilkins; 1994.include subcarinal (level 7), aortopulmonary (level 5), parae-sophageal (level 8),


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Surgery_Schwartz. from Shields TW: General Thoracic Surgery, 4th ed. Baltimore, MD: Lippincott Williams & Wilkins; 1994.include subcarinal (level 7), aortopulmonary (level 5), parae-sophageal (level 8), and inferior pulmonary ligament (level 9) as well as paratracheal (level 4).141 Technical expertise in these modalities should be pursued by thoracic and general surgeons.Historically, needle biopsies of anterior mediastinal masses were reportedly sensitive and specific for most carcinomatous tumors, but there were questions regarding accuracy for diagnos-ing lymphomas.142 However, advances in cytopathology as well as needle biopsy technology have substantially improved diag-nostic accuracy such that most centers are reporting yields rang-ing from 75% to 80% for the diagnosis of lymphoma as well. To achieve maximal diagnostic yield for mediastinal masses sug-gestive of a lymphoma, it is necessary to obtain multiple fine-needle aspirates, preferably with immediate onsite rapid cytologic analysis to


Surgery_Schwartz

Surgery_Schwartz. maximal diagnostic yield for mediastinal masses sug-gestive of a lymphoma, it is necessary to obtain multiple fine-needle aspirates, preferably with immediate onsite rapid cytologic analysis to confirm sampling of the target tissue and adequate cellularity. This also facilitates processing of the sample to ensure that proper studies for lymphoma, including flow cytometry, are obtained. If the needle biopsy is inconclusive, surgical biopsy can be performed.143,144 If the lesion is accessible by CT-guided or EUS-guided core-needle biopsy, intraoperative frozen section or immediate cytologic smear of a core biopsy can also be per-formed. Currently, core-needle biopsy with EBUS is not possible. The authors perform their own endobronchial, endoscopic, and CT-guided transbronchial and transthoracic biopsies, and in our experience, lack of cellularity in the aspirate is readily apparent. In general, plans to proceed with surgical biopsy are made in combination with the image-guided


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Surgery_Schwartz. transthoracic biopsies, and in our experience, lack of cellularity in the aspirate is readily apparent. In general, plans to proceed with surgical biopsy are made in combination with the image-guided aspiration and, as such, are performed in the same setting. This enables the authors to avoid a more invasive surgical procedure when FNA or core-needle biopsy is sufficient without contributing to delays in diagnosis by having multiple attempts from multiple providers (such as inter-ventional radiology and pulmonology) before involvement of the surgeon in the diagnostic workup.Table 19-28Signs and symptoms suggestive of various diagnoses in the setting of a mediastinal massDIAGNOSISHISTORY AND PHYSICAL FINDINGSCOMPARTMENT LOCATION OF MASSLymphomaNight sweats, weight loss, fatigue, extrathoracic adenopathy, elevated erythrocyte sedimentation rate or C-reactive protein level, leukocytosisAny compartmentThymoma with myasthenia gravisFluctuating weakness, early fatigue, ptosis,


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Surgery_Schwartz. extrathoracic adenopathy, elevated erythrocyte sedimentation rate or C-reactive protein level, leukocytosisAny compartmentThymoma with myasthenia gravisFluctuating weakness, early fatigue, ptosis, diplopiaAnteriorMediastinal granulomaDyspnea, wheezing, hemoptysisVisceral (middle)Germ cell tumorMale gender, young age, testicular mass, elevated levels of human chorionic gonadotropin and/or α-fetoproteinAnteriorBrunicardi_Ch19_p0661-p0750.indd 72901/03/19 7:01 PM 730SPECIFIC CONSIDERATIONSPART IITable 19-29Nuclear imaging relevant to the mediastinumRADIOPHARMACEUTICAL, RADIONUCLIDE, OR RADIOCHEMICALLABELDISEASE OF INTERESTIodine131I, 123IRetrosternal goiter, thyroid cancerMonoclonal antibodies111In, 99mTcNSCLC, colon and breast cancer, prostate cancer metastasesOctreotide111InAmine precursor uptake decarboxylation tumors: carcinoid, gastrinoma, insulinoma, small cell lung cancer, pheochromocytoma, glucagonoma, medullary thyroid carcinoma, paragangliomaGallium67GaLymphoma, NSCLC,


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Surgery_Schwartz. uptake decarboxylation tumors: carcinoid, gastrinoma, insulinoma, small cell lung cancer, pheochromocytoma, glucagonoma, medullary thyroid carcinoma, paragangliomaGallium67GaLymphoma, NSCLC, melanomaSestamibi99mTcMedullary thyroid carcinoma, nonfunctional papillary or follicular thyroid carcinoma, Hürthle cell thyroid carcinoma, parathyroid adenoma or carcinomaThallium201TlSee sestamibiMIBG131I, 123IPheochromocytoma, neuroblastoma; see also octreotideFluorodeoxyglucose18FGeneral oncologic imaging, breast and colon cancer, melanomaAbbreviations: MIGB = metaiodobenzylguanidine; NSCLC = non–small cell lung cancer.Reproduced with permission from Pearson FG, Cooper JD, Deslauriers J, et al: Thoracic Surgery, 2nd ed. New York, NY: Elsevier/Churchill Livingstone; 2002.Surgical Biopsies and Resection of Mediastinal MassesFor tumors of the mediastinum that are not amenable to an endo-scopic or CT-guided needle biopsy or that do not yield sufficient tissue for diagnosis, a surgical biopsy is


Surgery_Schwartz

Surgery_Schwartz. of Mediastinal MassesFor tumors of the mediastinum that are not amenable to an endo-scopic or CT-guided needle biopsy or that do not yield sufficient tissue for diagnosis, a surgical biopsy is indicated. The defini-tive approach to a surgical biopsy of the anterior mediastinum is through a median sternotomy. At the time of sternotomy, if the lesion is easily resectable, it should be completely removed. Given the invasiveness of the procedure and the inability in some patients to obtain a definitive diagnosis by frozen section, less invasive procedures are preferable if the lesion is large or if the CT scan or history suggests that surgery is not the best definitive treatment.Masses in the paratracheal region are easily biopsied by mediastinoscopy. For tumors of the anterior or posterior medi-astinum, a left or right VATS approach often allows safe and adequate surgical biopsies. In some patients, an anterior medi-astinotomy (i.e., Chamberlain procedure) may be ideal for an anterior


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Surgery_Schwartz. medi-astinum, a left or right VATS approach often allows safe and adequate surgical biopsies. In some patients, an anterior medi-astinotomy (i.e., Chamberlain procedure) may be ideal for an anterior tumor or a tumor with significant parasternal extension. Before a surgical biopsy is pursued, a discussion should be held with the pathologist regarding routine histologic assessment, spe-cial stains and markers, and requirements for lymphoma workup.Surgical resection using minimally invasive approaches, including video-assisted and robotic thoracoscopic surgery and transcervical, are now routine for the vast majority of middle and posterior tumors and for moderate sized (<5 to 6 cm) anterior mediastinal tumors.145-148 Outcomes comparing VATS to open thymectomy in patients with myasthenia gravis without thymoma were prospectively evaluated by Chang and colleagues in 2005, and no differences were seen in terms of response to therapy and recurrence of symptoms. Pain scores were


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Surgery_Schwartz. gravis without thymoma were prospectively evaluated by Chang and colleagues in 2005, and no differences were seen in terms of response to therapy and recurrence of symptoms. Pain scores were significantly better in the VATS approach.149 These reports and others support application of VATS for the majority of ante-rior mediastinal masses.Other minimally invasive approaches are under study. For example, good results have been reported using a cervical incision with a sternal retractor for thymus removal. The upward lift allows the surgeon reasonable access to the anterior medi-astinum and has proven adequate in some centers for definitive resection of the thymus gland for myasthenia gravis.150For larger anterior mediastinal masses or in centers where expertise in thoracoscopy is not available, median sternotomy and thoracotomy remain excellent options for resection of ante-rior mediastinal masses. Occasionally, a lateral thoracotomy with sternal extension (hemi-clamshell) provides


Surgery_Schwartz

Surgery_Schwartz. median sternotomy and thoracotomy remain excellent options for resection of ante-rior mediastinal masses. Occasionally, a lateral thoracotomy with sternal extension (hemi-clamshell) provides excellent exposure for extensive mediastinal tumors that have a lateral component.Most surgeons would agree that if a larger anterior medi-astinal tumor is seen or malignancy is suspected, a median ster-notomy with a more radical resection should be performed.Mediastinal NeoplasmsThymic Hyperplasia. Diffuse thymic hyperplasia was first described in children after successful chemotherapy for lym-phoma. It has now been described in adults and is referred to as “rebound thymic hyperplasia.”151 It is most frequently reported after chemotherapy for lymphoma or germ cell tumors. Ini-tially, atrophy of the thymic gland is seen with subsequent thymic gland enlargement, which can be dramatic. The usual time course for thymic hyperplasia is about 9 months after ces-sation of chemotherapy (range 2 weeks to


Surgery_Schwartz

Surgery_Schwartz. thymic gland is seen with subsequent thymic gland enlargement, which can be dramatic. The usual time course for thymic hyperplasia is about 9 months after ces-sation of chemotherapy (range 2 weeks to 12 months). Benign hyperplasia must be clearly distinguished from recurrent lym-phoma or germ cell tumors, which may be difficult since thymic hyperplasia is dramatic in some patients; careful follow-up with serial CT scans is the minimum requirement. The role of PET scanning is unclear. Thymic hyperplasia is a known cause of false-positive PET scans; in many patients, CT scan will show a triangular soft tissue density in the retrosternal space that has a characteristic bilobed anatomic appearance consistent with thymus gland.152 In addition, a low standardized uptake value of tracer on PET scan suggests a benign tumor.153 Biopsies may be required if the clinical index of suspicion is high.Brunicardi_Ch19_p0661-p0750.indd 73001/03/19 7:01 PM


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Surgery_Schwartz. CHAPTER 19731CHEST WALL, LUNG, MEDIASTINUM, AND PLEURAThymoma. While it is the most frequently encountered neo-plasm of the anterior mediastinum in adults (seen most fre-quently between 40 and 60 years of age), thymoma is rare in children. Between 10% and 50% of patients with thymoma will have symptoms suggestive of myasthenia gravis or have circulating antibodies to acetylcholine receptor, but less than 10% of patients with myasthenia gravis have a thymoma. Most patients with thymoma are asymptomatic. Thymectomy leads to improvement or resolution of symptoms of myasthe-nia gravis in only about 25% of patients with thymomas. In contrast, in patients with myasthenia gravis and no thymoma, thymectomy results are superior: up to 50% of patients have a complete remission, and 90% improve. In 5% of patients with thymomas, other paraneoplastic syndromes, including red cell aplasia, hypogammaglobulinemia, systemic lupus erythema-tosus, Cushing’s syndrome, or SIADH, may be present. Large


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Surgery_Schwartz. In 5% of patients with thymomas, other paraneoplastic syndromes, including red cell aplasia, hypogammaglobulinemia, systemic lupus erythema-tosus, Cushing’s syndrome, or SIADH, may be present. Large thymic tumors may present with symptoms related to a mass effect, which may include cough, chest pain, dyspnea, or SVC syndrome.The diagnosis may be suspected based on CT scan and history, but imaging alone is not diagnostic. In most centers, the diagnosis is made after surgical resection because of the rela-tive difficulty of obtaining a needle biopsy and the likelihood that removal will ultimately be recommended. Biopsy should be avoided in cases where imaging is highly suggestive of thy-moma. In most patients, the distinction between lymphomas and thymomas can be made on CT scan since most lymphomas have marked lymphadenopathy and thymomas most frequently appear as a solitary encapsulated mass. PET scan may have a role in differentiating thymic cancer from thymoma, as thymic cancer


Surgery_Schwartz

Surgery_Schwartz. lymphomas have marked lymphadenopathy and thymomas most frequently appear as a solitary encapsulated mass. PET scan may have a role in differentiating thymic cancer from thymoma, as thymic cancer tends to be very FDG avid.153 In addition, PET scan may facilitate identification of low-risk and minimally invasive thy-moma; a standardized uptake value (SUV) <5 was associated with Masaoka stage I or II thymoma, whereas invasive thymoma and mediastinal lymphoma were more likely when the SUV was >5.154 In cases where the diagnosis is unclear, transmediastinal, not transpleural, CT-guided FNA biopsy has a diagnostic sen-sitivity of 87% and a specificity of 95% in specialized centers.The most commonly accepted staging system for thymoma is that of Masaoka.155 It is based on the presence or absence of gross or microscopic invasion of the capsule and of surrounding structures, as well as on the presence or absence of metastases (Table 19-30). Histologically, thymomas are characterized by a


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Surgery_Schwartz. of gross or microscopic invasion of the capsule and of surrounding structures, as well as on the presence or absence of metastases (Table 19-30). Histologically, thymomas are characterized by a mixture of epithelial cells and mature lymphocytes. Grossly, many thymomas remain well encapsulated. Even those with capsular invasion often lack histologic features of malignancy; they appear cytologically benign and identical to early-stage tumors. This lack of classic cellular features of malignancy is why most pathologists use the term “thymoma” or “invasive thymoma” rather than “malignant thymoma.” Thymic tumors with malignant cytologic features are classified separately and referred to as “thymic carcinoma.”The definitive treatment for thymoma is complete surgical removal; local recurrence rates and survival vary according to stage (Fig. 19-47). In centers with significant experience with VATS procedures, thymoma is not a contraindication to VATS approach, provided the principles of


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Surgery_Schwartz. rates and survival vary according to stage (Fig. 19-47). In centers with significant experience with VATS procedures, thymoma is not a contraindication to VATS approach, provided the principles of resection are adhered to, such as a complete resection without disrupting the capsule.156 Otherwise, resection is generally accomplished by median ster-notomy with extension to hemi-clamshell in more advanced cases. Even advanced tumors with local invasion of resectable structures such as the pericardium, SVC, or innominate vessels should be considered for resection with reconstruction.A multidisciplinary approach to nonresectable and more advanced lesions (stage ≥II) is mandatory to optimize patient care. The goal for surgical resection should be complete exci-sion of the mass with total thymectomy. All contiguous and noncontiguous disease is removed at the same setting; this may include resection of the pericardium or pleura, adjacent adher-ent lung, phrenic nerve, major vascular


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Surgery_Schwartz. thymectomy. All contiguous and noncontiguous disease is removed at the same setting; this may include resection of the pericardium or pleura, adjacent adher-ent lung, phrenic nerve, major vascular structures, and pleural metastasis. Bilateral phrenic nerve resection should be avoided, however, due to the major respiratory morbidity associated with bilateral paralyzed hemidiaphragms.The role of adjuvant or neoadjuvant therapies for advanced-stage tumors remains unclear. Traditionally, stage II thymomas have been treated by complete surgical resection fol-lowed by mediastinal radiation, but randomized trials have not been done. A recent retrospective review of a single-institution series of stage II thymoma patients showed no difference in survival or local recurrence after complete surgical resection alone, as compared with surgical resection with radiotherapy. Advanced thymomas have been shown to respond to platinum-based chemotherapy and to corticosteroids.157 One summary of


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Surgery_Schwartz. surgical resection alone, as compared with surgical resection with radiotherapy. Advanced thymomas have been shown to respond to platinum-based chemotherapy and to corticosteroids.157 One summary of chemotherapy trials showed an overall response rate of about 70%. Cisplatin/doxorubicin-based regimens appear to yield the best results. The combination radiotherapy and chemotherapy for local progression appears to prolong survival in some small series.158 Radiation therapy in surgically resected stage III thy-moma is likely beneficial in extending disease-specific survival; an analysis of the Surveillance, Epidemiology, and End Results (SEER) database identified 476 patients with stage III thymoma treated with primary surgery. Postoperative radiation was given Table 19-30Masaoka staging system for thymomaStage IEncapsulated tumor with no gross or microscopic evidence of capsular invasionStage IIGross capsular invasion or invasion into the mediastinal fat or pleura or microscopic capsular


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Surgery_Schwartz. for thymomaStage IEncapsulated tumor with no gross or microscopic evidence of capsular invasionStage IIGross capsular invasion or invasion into the mediastinal fat or pleura or microscopic capsular invasionStage IIIGross invasion into the pericardium, great vessels, or lungStage IVAPleural or pericardial disseminationStage IVBLymphogenous or hematogenous metastasis1.0.8.6.4.20Proportion surviving02010YearsP=.002Stage IStage IVStage IIIStage IIFigure 19-47. Stage-specific survival for thymomas.Brunicardi_Ch19_p0661-p0750.indd 73101/03/19 7:01 PM 732SPECIFIC CONSIDERATIONSPART IIFigure 19-48. Massive thymolipoma that was asymptomatic in an 18-year-old female.to 322 patients with a significant improvement in survival (127 months compared to 105 months, P = .038) despite the fact that these patients were more likely to have had debulking rather than curative resection. In multivariate analysis, disease-specific survival was better in the adjuvant radiation group.159 There-fore, it is


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Surgery_Schwartz. patients were more likely to have had debulking rather than curative resection. In multivariate analysis, disease-specific survival was better in the adjuvant radiation group.159 There-fore, it is imperative that all patients with thymomas undergo a thorough evaluation for potential resection. Current guidelines recommend radiation for patients with unresectable thymoma who have failed induction chemotherapy or for patients with incompletely resected invasive thymoma or thymic cancer. Planning the radiation ports requires input from the surgeon; it is important for the surgeon to carefully document areas of adherence between the thymoma and adjacent structures during the operation, with clips or other radiopaque markers placed to guide radiation therapy postoperatively. Extracapsular extension and positive surgical margins should be noted by the pathologist and correlated anatomically so that the surgeon and radiation oncologist can ensure appropriate radiation treatment.Thymic


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Surgery_Schwartz. extension and positive surgical margins should be noted by the pathologist and correlated anatomically so that the surgeon and radiation oncologist can ensure appropriate radiation treatment.Thymic Carcinoma. Thymic carcinomas are unequivocally malignant at the microscopic level. Suster and Rosai classified thymic carcinomas into low-grade and high-grade tumors.160 Low-grade tumors are well differentiated with squamous cell, mucoepidermoid, or basaloid features. High-grade thymic car-cinomas include those with lymphoepithelial, small cell neu-roendocrine, sarcomatoid, clear cell, and undifferentiated or anaplastic features. Care must be taken to differentiate thymic carcinoma from lung cancer metastatic to the thymus gland as the histologic features can be similar between the two. Com-pared with thymomas, they are a more heterogeneous group of malignancies with a propensity for early local invasion and widespread metastases. Malignant pleural and pericardial effu-sions occur


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Surgery_Schwartz. Com-pared with thymomas, they are a more heterogeneous group of malignancies with a propensity for early local invasion and widespread metastases. Malignant pleural and pericardial effu-sions occur frequently.Five-year survival rates are between 30% and 50%. Com-plete resection is occasionally curative and leads to improved survival, but most thymic carcinomas will recur and are refractory to chemotherapy.157 Management, therefore, depends on the completeness of the resection. Postoperative care includes radiation therapy, guided by residual gross disease or microscopically positive margins from the resection specimen. Chemotherapy may also be given, with carboplatin/paclitaxel recommended based on the best response rates with the least toxicity in clinical trials. The prognosis of patients with thymic cancer remains poor.Thymolipoma. Thymolipomas are rare benign tumors that may grow to a very large size prior to diagnosis. On CT scan, their appearance can be dramatic, with a


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Surgery_Schwartz. patients with thymic cancer remains poor.Thymolipoma. Thymolipomas are rare benign tumors that may grow to a very large size prior to diagnosis. On CT scan, their appearance can be dramatic, with a characteristic fat den-sity dotted by islands of soft tissue density representing islands of thymic tissue (Fig. 19-48). Thymolipomas are generally well-encapsulated, soft, and pliable masses that do not invade surrounding structures. Resection is recommended for large masses.Neurogenic Tumors. Most neurogenic tumors of the medi-astinum arise from the cells of the nerve sheath, from ganglion cells, or from the paraganglionic system (Table 19-31). The incidence, cell types, and risk of malignancy strongly correlate with patient age. Tumors of nerve sheath origin predominate in adults. Most present as asymptomatic incidental findings, and most are benign. In children and young adults, tumors of the autonomic ganglia predominate, with up to two-thirds being malignant.161Nerve Sheath


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Surgery_Schwartz. Most present as asymptomatic incidental findings, and most are benign. In children and young adults, tumors of the autonomic ganglia predominate, with up to two-thirds being malignant.161Nerve Sheath Tumors. Nerve sheath tumors account for 20% of all mediastinal tumors. More than 95% of nerve sheath tumors are benign neurilemomas or neurofibromas. Malignant neurosarcomas are much less common.Neurilemoma. Neurilemomas, also called schwannomas, arise from Schwann cells in intercostal nerves. They are firm, well encapsulated, and generally benign. Two characteristic histo-logic components are referred to as Antoni type A and Antoni type B regions. Antoni type A regions contain compact spindle Brunicardi_Ch19_p0661-p0750.indd 73201/03/19 7:01 PM


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Surgery_Schwartz. CHAPTER 19733CHEST WALL, LUNG, MEDIASTINUM, AND PLEURAFigure 19-49. Magnetic resonance image of a neurogenic tumor with extension into the spinal canal via the foramen, giving a typical dumbbell appearance.Table 19-31Classification of neurogenic tumors of the mediastinumTUMOR ORIGINBENIGNMALIGNANTNerve sheathNeurilemoma, neurofibroma, melanotic schwannoma, granular cell tumorNeurofibrosarcomaGanglion cellGanglioneuromaGanglioneuroblastoma, neuroblastomaParaganglionic cellChemodectoma, pheochromocytomaMalignant chemodectoma, malignant pheochromocytomaReproduced with permission from Pearson FG, Cooper JD, Deslauriers J, et al: Thoracic Surgery, 2nd ed. New York, NY: Elsevier/Churchill Livingstone; 2002.cells with twisted nuclei and nuclear palisading. Antoni type B regions contain loose and myxoid connective tissue with hap-hazard cellular arrangement. These characteristics distinguish neurilemoma from malignant fibrosarcomatous tumors, which lack encapsulation and have no Antoni


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Surgery_Schwartz. and myxoid connective tissue with hap-hazard cellular arrangement. These characteristics distinguish neurilemoma from malignant fibrosarcomatous tumors, which lack encapsulation and have no Antoni features. If routine CT scan suggests extension of a neurilemoma into the intervertebral foramen, MRI is used to evaluate the extent of this “dumbbell” configuration (Fig. 19-49). Such a configuration may lead to cord compression and paralysis and requires a more complex surgical approach. Resection is recommended; VATS has been established as safe and effective for simple and, in experienced centers, even the more complex operations.162 It is reasonable to follow small, asymptomatic paravertebral tumors in older patients or in patients at high risk for surgery. In children, ganglioneuroblastomas or neuroblastomas are more common; therefore, all neurogenic tumors should be completely resected.Neurofibroma. Neurofibromas consist of both nerve sheath and nerve cells and account for up to 25%


Surgery_Schwartz

Surgery_Schwartz. or neuroblastomas are more common; therefore, all neurogenic tumors should be completely resected.Neurofibroma. Neurofibromas consist of both nerve sheath and nerve cells and account for up to 25% of nerve sheath tumors. Up to 40% of patients with mediastinal fibromas have general-ized neurofibromatosis (von Recklinghausen’s disease). About 70% of neurofibromas are benign, but malignant degeneration to neurofibrosarcoma occurs in 25% to 30% of patients.163 The risk of malignant degeneration increases with advancing age, von Recklinghausen’s disease, and exposure to previous radia-tion. Neurofibrosarcomas carry a poor prognosis because of rapid growth and aggressive local invasion along nerve bun-dles. Complete surgical resection is the mainstay of treatment. Adjuvant radiotherapy or chemotherapy does not confer a sig-nificant benefit, but may be added if complete resection is not possible.164 The 5-year survival rate is 53%, but it drops to 16% in patients with neurofibromatosis or


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Surgery_Schwartz. does not confer a sig-nificant benefit, but may be added if complete resection is not possible.164 The 5-year survival rate is 53%, but it drops to 16% in patients with neurofibromatosis or with large tumors (>5 cm).Ganglion Cell Tumors Ganglion cell tumors (ganglioneuro-mas, ganglioneuroblastomas, and neuroblastomas) arise from the sympathetic chain or from the adrenal medulla.1. Ganglioneuroma. Well-differentiated, benign tumors char-acterized histologically by well-differentiated ganglion cells with a background of Schwann cells, these are most often found incidentally in asymptomatic young adults. Diarrhea related to secretion of a vasoactive intestinal peptide has been described in some patients. These tumors have a pro-pensity for intraspinal canal extension, although they remain well-encapsulated; complete resection is curative, with a low risk of local recurrence.Brunicardi_Ch19_p0661-p0750.indd 73301/03/19 7:01 PM 734SPECIFIC CONSIDERATIONSPART II2. Ganglioneuroblastoma.


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Surgery_Schwartz. complete resection is curative, with a low risk of local recurrence.Brunicardi_Ch19_p0661-p0750.indd 73301/03/19 7:01 PM 734SPECIFIC CONSIDERATIONSPART II2. Ganglioneuroblastoma. Ganglioneuroblastomas contain a mixture of benign ganglion cells and malignant neuro-blasts. The distribution of these cells within the tumor is predictive of the clinical course. The nodular pattern has a high incidence of metastatic disease, whereas the diffuse pattern rarely metastasizes. Gross examination typically reveals encapsulated tumor; histologically, there are focal calcifications around regions of neuroblasts. Ganglioneu-roblastomas arise most frequently in infants and children <3 years old. The majority are resectable, with 80% 5-year survival.3. Neuroblastoma. Highly malignant, neuroblastomas are the most common extracranial solid malignancy of childhood. The primary site is intrathoracic malignancy in 14%; exten-sion into the spinal canal and osseous invasion commonly present. These


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Surgery_Schwartz. are the most common extracranial solid malignancy of childhood. The primary site is intrathoracic malignancy in 14%; exten-sion into the spinal canal and osseous invasion commonly present. These thoracic tumors are not as recalcitrant to chemotherapy and surgical resection as other chest malig-nancies; they are more likely to be resectable, with less inva-sion of surrounding organs. More than half occur in children under 2 years old; 90% arise within the first decade of life, and thus, these malignancies are discussed in more detail in Chapter 39.Paraganglionic Tumors. Paraganglionic tumors arising in the thoracic cavity include chemodectomas and pheochromo-cytomas. Only 10% of all pheochromocytomas are located in an extra-adrenal site. Intrathoracic pheochromocytomas are one of the rarest tumors.Approximately 10% of thoracic pheochromocytomas are malignant, a rate similar to that of adrenal tumors. The most common thoracic location is within the costovertebral sulcus, but


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Surgery_Schwartz. the rarest tumors.Approximately 10% of thoracic pheochromocytomas are malignant, a rate similar to that of adrenal tumors. The most common thoracic location is within the costovertebral sulcus, but paraganglionic tumors also arise within the visceral com-partment of the mediastinum. These catecholamine-producing lesions can lead to life-threatening hemodynamic problems, so complete removal is important. Diagnosis is generally confirmed by measuring elevated levels of urinary catecholamines and their metabolites. Localization is by CT scan, aided by MIBG scintigraphy. Preoperative care includes αand β-adrenergic blockade to prevent intraoperative malignant hypertension and arrhythmias. These tumors tend to be highly vascular and should be approached with care. Chemodectomas are rare tumors that may be located around the aortic arch, vagus nerves, or aor-ticosympathetics. They rarely secrete catecholamines and are malignant in up to 30% of patients.Lymphoma. Overall, lymphomas are the


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Surgery_Schwartz. that may be located around the aortic arch, vagus nerves, or aor-ticosympathetics. They rarely secrete catecholamines and are malignant in up to 30% of patients.Lymphoma. Overall, lymphomas are the most common malig-nancy of the mediastinum. In about 50% of patients who have both Hodgkin’s and non-Hodgkin’s lymphoma, the mediasti-num may be the primary site. The anterior compartment is most commonly involved, with occasional involvement of the mid-dle compartment and hilar nodes. The posterior compartment is rarely involved. Chemotherapy and/or radiation results in a cure rate of up to 90% for patients with early-stage Hodgkin’s disease and up to 60% with more advanced stages.Mediastinal Germ Cell Tumors. Germ cell tumors are uncom-mon neoplasms, but they are the most common malignancy in young men 15 to 35 years of age. Most germ cell tumors are gonadal in origin; primary mediastinal germ cell tumors com-prise less than 5% of all germ cell tumors and less than 1% of all mediastinal


Surgery_Schwartz

Surgery_Schwartz. young men 15 to 35 years of age. Most germ cell tumors are gonadal in origin; primary mediastinal germ cell tumors com-prise less than 5% of all germ cell tumors and less than 1% of all mediastinal tumors (usually occurring in the anterior com-partment). If a malignant mediastinal germ cell tumor is found, it is important to exclude a gonadal primary tumor. Primary mediastinal germ cell tumors (including teratomas, seminomas, and nonseminomatous malignant germ cell tumors) are a het-erogeneous group of benign and malignant neoplasms thought to originate from primitive pluripotent germ cells “misplaced” in the mediastinum during embryonic development. Previ-ously, most mediastinal germ cell tumors were thought to be metastatic. However, two lines of evidence suggest that many mediastinal germ cell tumors are primary, developing from plu-ripotent primordial germ cells in the mediastinum: (a) several autopsy series showed that patients with extragonadal sites of germ cell tumors,


Surgery_Schwartz

Surgery_Schwartz. germ cell tumors are primary, developing from plu-ripotent primordial germ cells in the mediastinum: (a) several autopsy series showed that patients with extragonadal sites of germ cell tumors, presumed previously to have originated from the gonads, had no evidence of an occult primary tumor or of any residual scar of the gonads, even after an exhaustive search; and (b) patients treated by surgery or radiation for their medi-astinal germ cell tumors had long-term survival with no late testicular recurrences.165About one-third of all primary mediastinal germ cell tumors are seminomatous. Two-thirds are nonseminomatous tumors or teratomas. Treatment and prognosis vary consider-ably within these two groups. Mature teratomas are benign and can generally be diagnosed by the characteristic CT findings of multilocular cystic tumors, encapsulated with combinations of fluid, soft tissue, calcium, and/or fat attenuation in the anterior compartment. FNA biopsy alone may be diagnostic for


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Surgery_Schwartz. CT findings of multilocular cystic tumors, encapsulated with combinations of fluid, soft tissue, calcium, and/or fat attenuation in the anterior compartment. FNA biopsy alone may be diagnostic for semi-nomas, usually with normal serum markers, including hCG and AFP. In 10% of seminomas, hCG levels may be slightly ele-vated. FNA findings, along with high hCG and AFP levels, can accurately diagnose nonseminomatous tumors. If the diagnosis remains uncertain after assessment of FNA findings and serum marker levels, then core-needle biopsies or surgical biopsies may be required. Thoracoscopy is the most frequent diagnostic surgical approach.1. Seminoma. Most patients with seminomas have advanced disease at the time of diagnosis and present with symptoms of local compression, including SVC syndrome, dyspnea, or chest discomfort. With advanced disease, the preferred treatment is combination cisplatin-based chemotherapy regimens with bleomycin and either etoposide or vinblas-tine. Complete


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Surgery_Schwartz. dyspnea, or chest discomfort. With advanced disease, the preferred treatment is combination cisplatin-based chemotherapy regimens with bleomycin and either etoposide or vinblas-tine. Complete responses have been reported in over 75% of patients treated with these regimens. Surgical resection may be curative for small asymptomatic seminomas that are found incidentally with screening CT scans. Surgical resection of residual masses after chemotherapy may be indicated.2. Nonseminomatous germ cell tumors. Nonseminomatous germ cell tumors include embryonal cell carcinomas, cho-riocarcinomas, endodermal sinus tumors, and mixed types. They are often bulky, irregular tumors of the anterior medi-astinum with areas of low attenuation on CT scan because of necrosis, hemorrhage, or cyst formation. Frequently, adjacent structures have been involved, with metastases to regional lymph nodes, pleura, and lungs. Lactate dehy-drogenase (LDH), AFP, and hCG levels are frequently elevated. Chemotherapy is


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Surgery_Schwartz. adjacent structures have been involved, with metastases to regional lymph nodes, pleura, and lungs. Lactate dehy-drogenase (LDH), AFP, and hCG levels are frequently elevated. Chemotherapy is the preferred treatment and includes combination therapy with cisplatin, bleomycin, and etoposide, followed by surgical resection of residual disease. With this regimen, survival is 67% at 2 years and 60% at 5 years. Surgical resection of residual masses is indicated, as it may guide further therapy. Up to 20% of residual masses contain additional tumors; in another 40%, mature teratomas; and the remaining 40%, fibrotic tissue. Brunicardi_Ch19_p0661-p0750.indd 73401/03/19 7:01 PM


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Surgery_Schwartz. CHAPTER 19735CHEST WALL, LUNG, MEDIASTINUM, AND PLEURAIt is important to note that oxygen toxicity can occur in patients who have been exposed to bleomycin; high lev-els of oxygen supplementation in the perioperative setting should be avoided in these patients as respiratory failure and death can ensue.166 Factors independently predictive of survival after induction chemotherapy followed by resec-tion are elevated serum tumor markers after resection, postchemotherapy pathologic findings (complete necrosis vs. teratoma), and persistent germ cell or non–germ cell cancer in the pathologic specimen.1663. Teratoma. Teratomas are the most common type of medi-astinal germ cell tumors, accounting for 60% to 70% of mediastinal germ cell tumors. They contain two or three embryonic layers that may include teeth, skin, and hair (ectodermal), cartilage and bone (mesodermal), or bron-chial, intestinal, or pancreatic tissue (endodermal). Ther-apy for mature, benign teratomas is surgical resection,


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Surgery_Schwartz. include teeth, skin, and hair (ectodermal), cartilage and bone (mesodermal), or bron-chial, intestinal, or pancreatic tissue (endodermal). Ther-apy for mature, benign teratomas is surgical resection, which confers an excellent prognosis. Rarely, teratomas may contain a focus of carcinoma; these malignant tera-tomas (or teratocarcinomas) are locally aggressive. Often diagnosed at an unresectable stage, they respond poorly to chemotherapy and in a limited manner to radiotherapy; prognosis is uniformly poor.Mediastinal CystsBenign cysts account for up to 25% of mediastinal masses and are the most frequently occurring mass in the middle medias-tinal compartment. A CT scan showing characteristic features of near water density in a typical location is virtually 100% diagnostic.1671. Pericardial cyst. Usually asymptomatic and detected inci-dentally in the right costophrenic angle, pericardial cysts typically contain a clear fluid and are lined with a single layer of mesothelial cells. For


Surgery_Schwartz

Surgery_Schwartz. cyst. Usually asymptomatic and detected inci-dentally in the right costophrenic angle, pericardial cysts typically contain a clear fluid and are lined with a single layer of mesothelial cells. For most simple, asymptomatic pericardial cysts, observation alone is recommended. Sur-gical resection or aspiration may be indicated for complex cysts or large symptomatic cysts.2. Bronchogenic cyst. Developmental anomalies that occur during embryogenesis and occur as an abnormal budding of the foregut or tracheobronchial tree, bronchogenic cysts arise most often in the mediastinum just posterior to the carina or main stem bronchus. Approximately 15% occur within the pulmonary parenchyma. Thin-walled and lined with respiratory epithelium, they contain a protein-rich mucoid material and varying amounts of seromucous glands, smooth muscle, and cartilage. They may commu-nicate with the tracheobronchial tree. In adults, over half of all bronchogenic cysts are found incidentally during workup for an


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Surgery_Schwartz. of seromucous glands, smooth muscle, and cartilage. They may commu-nicate with the tracheobronchial tree. In adults, over half of all bronchogenic cysts are found incidentally during workup for an unrelated problem or during screening. The natural history of an incidentally diagnosed, asymptomatic bronchogenic cyst is unknown, but it is clear that many such cysts do not lead to clinical problems. In one study of young military personnel, 78% of all bronchogenic cysts found on routine CXRs were asymptomatic. However, in other reports with more comprehensive follow-up, up to 67% of adults with incidentally found bronchogenic cysts eventu-ally became symptomatic. Symptoms include chest pain, cough, dyspnea, and fever. If large (>6 cm) or symptomatic, resection is generally recommended since serious compli-cations may occur if the cyst becomes larger or infected. Complications include airway obstruction, infection, rup-ture, and, rarely, malignant transformation.168,169 Traditionally,


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Surgery_Schwartz. serious compli-cations may occur if the cyst becomes larger or infected. Complications include airway obstruction, infection, rup-ture, and, rarely, malignant transformation.168,169 Traditionally, complete removal of the cyst wall has been via posterolateral thoracotomy.170 Resection of infected cysts may be quite difficult because of dense adhesions; elective removal is often recommended before infection has a chance to occur. Thoracoscopic exploration and resec-tion are possible for small cysts with minimal adhesions. With increasing experience using video-assisted or robotic-assisted thoracoscopy, a greater proportion of these lesions are amenable to minimally invasive resection.3. Enteric cyst. Most clinicians agree that in contrast to bronchogenic cysts, esophageal cysts should be removed, regardless of the presence or absence of symptoms. Esopha-geal cysts have a propensity for serious complications sec-ondary to enlargement, leading to hemorrhage, infection, or perforation.


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Surgery_Schwartz. removed, regardless of the presence or absence of symptoms. Esopha-geal cysts have a propensity for serious complications sec-ondary to enlargement, leading to hemorrhage, infection, or perforation. Thus, surgical resection is the treatment of choice in both adults and children. As with bronchogenic cysts, experienced surgeons are approaching enteric cyst resections using minimally invasive techniques with great success.4. Thymic cyst. Generally asymptomatic, thymic cysts are often discovered incidentally. Simple cysts are of no con-sequence; however, the occasional cystic neoplasm must be ruled out. Cystic components occasionally are seen in patients with thymoma and Hodgkin’s disease.5. Ectopic endocrine glands. Up to 5% of all mediastinal masses are of thyroid origin; most are simple extensions of thyroid masses. Usually nontoxic, over 95% can be com-pletely resected through a cervical approach. True ectopic thyroid tissue of the mediastinum is rare. About 10% to 20% of abnormal


Surgery_Schwartz

Surgery_Schwartz. extensions of thyroid masses. Usually nontoxic, over 95% can be com-pletely resected through a cervical approach. True ectopic thyroid tissue of the mediastinum is rare. About 10% to 20% of abnormal parathyroid glands are found in the mediasti-num; most can be removed during exploration from a cervi-cal incision. In cases of true mediastinal parathyroid glands, thoracoscopic or open resection may be indicated. Location can generally be pinpointed by a combination of CT scan and Sestamibi scans.MediastinitisAcute Mediastinitis. Acute mediastinitis is a fulminant infec-tious process that spreads rapidly along the continuous fascial planes connecting the cervical and mediastinal compartments. Infections originate most commonly from esophageal perfora-tions, sternal infections, and oropharyngeal or neck infections, but a number of less common etiologic factors can lead to this deadly process (Table 19-32). Clinical signs and symptoms include fever, chest pain, dysphagia, respiratory


Surgery_Schwartz

Surgery_Schwartz. or neck infections, but a number of less common etiologic factors can lead to this deadly process (Table 19-32). Clinical signs and symptoms include fever, chest pain, dysphagia, respiratory distress, and cervical and upper thoracic subcutaneous crepitus. In severe cases, the clinical course can rapidly deteriorate to florid sepsis, hemodynamic instability, and death. Thus, a high index of sus-picion is required in the context of any infection with access to the mediastinal compartments.A chest CT scan illuminates the extent of spread and guides selection of the best approach to surgical drainage. Acute mediastinitis is a true surgical emergency; treatment must be instituted immediately and aimed at correcting the primary problem, such as the esophageal perforation or oro-pharyngeal abscess, and debridement and drainage of the spreading infectious process within the mediastinum, neck, pleura, and other tissue planes. Antibiotics, fluid resusci-tation, and other supportive measures are