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Surgery_Schwartz. 180918/02/19 4:34 PM 1810SPECIFIC CONSIDERATIONSPART IIradical local excision are adequate. Patients with IB tumors have deeper invasion but negative nodes and therefore carry an excellent prognosis. Stage II includes patients with local exten-sion and negative nodes and therefore carry a prognosis similar to other node-negative patients.Stage III disease includes patients with lymph node metas-tases, and stage IV disease is either locally advanced or distant metastasis. Treatment options for stage III and stage IV dis-ease include (a) chemoradiation followed by limited resection if needed; (b) radical vulvectomy; and (c) radical vulvectomy coupled with pelvic exenteration. External beam radiotherapy combined with radiosensitizing chemotherapy of cisplatin and 5-fluorouracil (5-FU) is emerging as the preferred initial management of advanced disease, followed by limited surgical resection of residual disease.88-90 Reconstruction of the vulva and groin, if needed, can be


Surgery_Schwartz

Surgery_Schwartz. (5-FU) is emerging as the preferred initial management of advanced disease, followed by limited surgical resection of residual disease.88-90 Reconstruction of the vulva and groin, if needed, can be accomplished using grafts and rota-tional or myocutaneous flaps depending on the size and type of defect.Inguinofemoral lymphadenectomy is indicated beyond clinical stage IA. Unilateral lymphadenectomy is recom-mended for lateralized lesions or bilateral for central lesions that cross the midline, or those involving the periclitoral area (Figs. 41-19 and 41-20). Complications of complete inguino-femoral lymphadenectomy include wound dehiscence or infec-tion and lymphedema. Sentinel lymph node biopsy (SLNB) is an alternative to inguinofemoral lymphadenectomy for selected patients with stage I or II disease and no palpable inguinofemo-ral nodes. SLNB appears to be effective in detecting inguino-femoral lymph node metastases without increasing the risk of groin recurrence while avoiding the


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Surgery_Schwartz. or II disease and no palpable inguinofemo-ral nodes. SLNB appears to be effective in detecting inguino-femoral lymph node metastases without increasing the risk of groin recurrence while avoiding the morbidities associated with complete inguinofemoral lymphadenectomy. Several prospec-tive studies support this approach.91,92 However, it is recognized that successful SLNB depends on operator experience. Surgeons with limited experience in SLNB (have performed fewer than 10 of these procedures) may choose to perform complete groin node dissection or use this procedure only for tumors that are less than 2 cm in size.Nodal failure in the groin and pelvis is difficult to treat successfully, and attention to primary management of these areas is key. Postoperative adjuvant inguinal and pelvic radio-therapy is indicated when inguinal lymph nodes are positive and is superior to pelvic lymphadenectomy, which has been largely abandoned. It is also indicated when the vulvectomy margins are


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Surgery_Schwartz. radio-therapy is indicated when inguinal lymph nodes are positive and is superior to pelvic lymphadenectomy, which has been largely abandoned. It is also indicated when the vulvectomy margins are positive or close positive for disease and further surgical management is not anatomically feasible.Vaginal CancerVaginal carcinoma is a rare gynecologic malignancy and accounts for about 3% of cancers affecting the female repro-ductive system.84 Squamous cell carcinomas account for 85% to 90% of cases; more than two-thirds of vaginal cancers are diagnosed in women 60 years of age or older. Risk factors are similar to other HPV-related cervical and vulvar cancers. Rare clear cell carcinoma of the vagina is associated to in utero expo-sure to diethylstilbestrol (DES), which is now largely of his-torical interest due to aging of the exposed cohort.93 Patients with vaginal cancer usually present with postmenopausal and/or postcoital bleeding and may also complain of vaginal discharge, vaginal


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Surgery_Schwartz. interest due to aging of the exposed cohort.93 Patients with vaginal cancer usually present with postmenopausal and/or postcoital bleeding and may also complain of vaginal discharge, vaginal mass, dysuria, hematuria, rectal bleeding, or pelvic pain, which may be indicative of advanced disease. Diagnosis is made via biopsy of suspicious lesions, which may require colposcopic guidance.85Figure 41-18. Extent of modified radical hemivulvectomy for stages I and II squamous cancer of the vulva.Superficial femoral nodesCribriformfasciaDeep femoral nodesFemoral a.Femoral n.Sartorius m.Iliopsoas m.FemurEpidermuslateralmedialAdductor longusPectineus m.Femoral v.Camper’s fasciaFigure 41-19. The anatomy of the inguinal triangle by cross-section.Pubic tubercleFemoral v.Sapheno-femoraljunctionFigure 41-20. Landmarks for choosing an incision for an inguinal lymphadenectomy.Brunicardi_Ch41_p1783-p1826.indd 181018/02/19 4:34 PM 1811GYNECOLOGYCHAPTER 41Vaginal cancer is staged clinically by pelvic


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Surgery_Schwartz. for choosing an incision for an inguinal lymphadenectomy.Brunicardi_Ch41_p1783-p1826.indd 181018/02/19 4:34 PM 1811GYNECOLOGYCHAPTER 41Vaginal cancer is staged clinically by pelvic exam, chest X-ray, cystoscopy, and proctoscopy (Table 41-7).94 Vaginal cancer spreads by local extension to adjacent pelvic structures, by lymphatic embolization to regional lymph nodes, and, less commonly, via the hematogenous route. Lymphatic drainage is complex, but in general, lesions in the upper vagina drain to the pelvic lymph nodes while lesions involving the lower third drain to the inguinofemoral lymph nodes.Stage I disease, involving the upper vagina, may be treated surgically or with intracavitary radiation therapy.86,87,95 Surgery consists of a radical hysterectomy, upper vaginectomy, and bilateral pelvic lymphadenectomy. Stage I disease in the mid to lower vagina is treated with radiation and concurrent chemo-therapy. External beam pelvic radiation is the mainstay of treat-ment for stages


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Surgery_Schwartz. pelvic lymphadenectomy. Stage I disease in the mid to lower vagina is treated with radiation and concurrent chemo-therapy. External beam pelvic radiation is the mainstay of treat-ment for stages II to IV and may be followed by intracavitary Table 41-7FIGO staging of vaginal carcinoma0Carcinoma in situ; intraepithelial neoplasia grade 3ITumor limited to the vaginal wallIITumor has involved the subvaginal tissue but has not extended to the pelvic wallIIITumor extends to the pelvic wallIVTumor has extended beyond the true pelvis or has involved the mucosa of the bladder or rectumIVATumor invades bladder and/or rectal mucosa and/or direct extension beyond the true pelvisIVBDistant metastasisand/or interstitial brachytherapy. Prognosis for treated early stage disease is excellent with more than 90% 5-year survival rates. Advanced stage disease, however, carries a poor progno-sis with only 15% to 40% 5-year survival rates.Cervical CancerGeneral Principles. There are over 12,000 new cases


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Surgery_Schwartz. 90% 5-year survival rates. Advanced stage disease, however, carries a poor progno-sis with only 15% to 40% 5-year survival rates.Cervical CancerGeneral Principles. There are over 12,000 new cases of cervical cancer and over 4000 cervical cancer deaths annually in the United States.96 It is a major killer worldwide causing 275,000 deaths annually.97 Risk factors for cervical squamous cell and adenocarcinoma, the two most common histologies, are largely related to acquisition of and immune response to carcinogenic subtypes of the HPV virus. Cervical screening is correlated with early identification and treatment of preinvasive disease.98 Cervical cancer is most commonly identified in women with long intervals between screenings, or with no prior screening. It is also associated with early age at first intercourse, multiple sexual partners, smoking, and oral contraceptive use.Early cervical cancer is usually asymptomatic, though irregu-lar or postcoital bleeding may be present,


Surgery_Schwartz

Surgery_Schwartz. early age at first intercourse, multiple sexual partners, smoking, and oral contraceptive use.Early cervical cancer is usually asymptomatic, though irregu-lar or postcoital bleeding may be present, particularly in more advanced disease. The diagnosis of cervical cancer is made by cervical biopsy, either of a gross lesion or a colposcopically-identified lesion. Cervical cancer is staged clinically due to the high disease burden in the developing world.99 Despite the prog-nostic value of clinical staging, in the developed world, surgical and radiologic staging is used to determine the extent of tumor spread and identify lymph node involvement. Lymph node metastasis is common and one of the most important prognostic factors in this disease, and positron emission tomography scans are useful in pretreatment planning and determination of radia-tion fields for women with locally advanced disease. Staging and management options are outlined in Table 41-8.7Table 41-82009 FIGO cervical cancer


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Surgery_Schwartz. in pretreatment planning and determination of radia-tion fields for women with locally advanced disease. Staging and management options are outlined in Table 41-8.7Table 41-82009 FIGO cervical cancer staging and management optionsSTAGEDESCRIPTIONOPTIONS FOR MANAGEMENT0Carcinoma in situAdenocarcinoma in situ: simple hysterectomy, may be followed for fertility preservation if all margins negative on coneSquamous cell carcinoma in situ: local excision with LEEP or cone or laser ablationIConfined to the cervixA1: Confined to the cervix, diagnosed only by microscopy with invasion of ≤3 mm in depth and lateral spread ≤7 mmA2: Confined to the cervix, diagnosed with microscopy with invasion of >3 mm and <5 mm with lateral spread ≤7 mmB1: Clinically visible lesion or greater than A2, ≤4 cm in greatest dimensionB2: Clinically visible lesion, >4 cm in greatest dimensionA1 and some A2: fertility preservation through large cone followed by close monitoring, followed by hysterectomyB1 and B2:


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Surgery_Schwartz. greatest dimensionB2: Clinically visible lesion, >4 cm in greatest dimensionA1 and some A2: fertility preservation through large cone followed by close monitoring, followed by hysterectomyB1 and B2: radical hysterectomy or chemoradiation; radical trachelectomy with uterine preservation for childbearing is under investigation for highly selected patients with small lesionsIIA1: Involvement of the upper two-thirds of the vagina, without parametrial invasion, ≤4 cm in greatest dimensionA2: >4 cm in greatest dimensionB: Parametrial involvementFor some IIA radical hysterectomy may be consideredIIA and B: chemoradiation is preferredIIIA. Involvement of the lower third of the vaginaB. Involvement of a parametria to the sidewall or obstruction of one or both ureters on imagingChemoradiationIVA. Local involvement of the bladder or rectumB. Distant metastasesA. ChemoradiationB. Chemotherapy with palliative radiation as indicatedData from Pecorelli S: Revised FIGO staging for carcinoma of the


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Surgery_Schwartz. Local involvement of the bladder or rectumB. Distant metastasesA. ChemoradiationB. Chemotherapy with palliative radiation as indicatedData from Pecorelli S: Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium, Int J Gynaecol Obstet. 2009 May;105(2):103-104.Brunicardi_Ch41_p1783-p1826.indd 181118/02/19 4:34 PM 1812SPECIFIC CONSIDERATIONSPART IIProcedures for Cervical Cancer Treatment. Certain cervical cancers that are confined to the cervix may be treated surgically. Very small lesions (less than 7 mm wide, less than 3 mm deep) with no LVSI may be treated with simple hysterectomy. In a woman who desires future fertility, a cone biopsy with negative surgical margins may be an acceptable alternative. Any tumor larger than this (larger than stage IA1) should be treated with radical hysterectomy or in special cases radical trachelectomy for fertility preservation. Some authors advocate a large cone biopsy with lymph node dissection for stage IA2 tumors in patients


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Surgery_Schwartz. with radical hysterectomy or in special cases radical trachelectomy for fertility preservation. Some authors advocate a large cone biopsy with lymph node dissection for stage IA2 tumors in patients who desire future fertility, though this recommenda-tion is somewhat controversial. Tumors that are greater than 4 cm in size are most often treated with chemoRT even if they Figure 41-21. Radical hysterectomy.BAUterusOvaryFallopian tubeCRound ligamentVesicouterinefoldUterinevesselsEDPararectalspaceLymphnodesParavesical spaceFExternal iliac vesselsInternal iliac arteryGHISuperior vesicalarteryUterine arteryare confined to the cervix, given the high likelihood of need for postoperative radiotherapy due to cervical risk factors.Radical Hysterectomy This procedure may be performed via laparotomy, or increasingly via a minimally invasive (laparo-scopic or robotic) approach.100 The key elements are dissection of the pelvic and periaortic nodes and the dissection of the para-metrium from the


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Surgery_Schwartz. or increasingly via a minimally invasive (laparo-scopic or robotic) approach.100 The key elements are dissection of the pelvic and periaortic nodes and the dissection of the para-metrium from the pelvic sidewall to allow en bloc removal with the uterus. The principle steps of an open procedure are demon-strated in Fig. 41-21. In contrast to a typical simple hysterectomy, the radical hysterectomy involves dissection much closer to the bowel, bladder, ureters, and great vessels, resulting in a higher complication rate to these organs. Additionally, disruption of the Brunicardi_Ch41_p1783-p1826.indd 181218/02/19 4:35 PM 1813GYNECOLOGYCHAPTER 41MUreterVaginaJKOvary and ligamentFallopian tubeUreterLUterosacralligamentFigure 41-21. (Continued)nerves supplying the bladder and the rectum, which traverse the cardinal and uterosacral ligaments, may result in temporary or long-term bladder and bowel dysfunction. Radical hysterecto-mies allow for the maintenance of the ovaries since the


Surgery_Schwartz

Surgery_Schwartz. which traverse the cardinal and uterosacral ligaments, may result in temporary or long-term bladder and bowel dysfunction. Radical hysterecto-mies allow for the maintenance of the ovaries since the incidence of metastases to this area is very low, providing a clear advantage of surgery over radiation therapy in the younger patient.Radical Trachelectomy Interest in fertility preservation with stages IA1 and 2, and stage IB1 lesions has led to the develop-ment of methods of radical trachelectomy with uterine preserva-tion. This procedure depends on an adequate blood supply to the uterus from the ovarian anastamoses, as the cervical portion is removed. The lower uterine segment closed with a cerclage and attached directly to the vaginal cuff. The rates of recurrence, pregnancy outcomes, and the best surgical candidates for this surgery are still under study,101 but there are sufficient numbers and experience, both obstetric and surgical, to suggest that this procedure is oncologically


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Surgery_Schwartz. the best surgical candidates for this surgery are still under study,101 but there are sufficient numbers and experience, both obstetric and surgical, to suggest that this procedure is oncologically safe and allows live births.Pelvic Exenteration for Recurrent Disease (Fig. 41-22) Cervical cancer recurrences after primary surgical management are treated with radiation. Surgery may be a consideration in selected patients with recurrent cervical cancer who have received maximal radiation therapy. If the recurrence is locally confined with no evidence of spread or metastatic disease, then pelvic exenteration may be considered. Attempted exenteration procedures are aborted intraoperatively if metastatic disease is found. Exenteration is tailored for the disease size and location and may be supralevator or extend below the levator ani muscle and require vulvar resection. Reconstruction of the pelvis may require a continent urinary pouch (if radiation enteritis is limited) or ileal conduit


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Surgery_Schwartz. or extend below the levator ani muscle and require vulvar resection. Reconstruction of the pelvis may require a continent urinary pouch (if radiation enteritis is limited) or ileal conduit and colostomy, as well as rebuilding of the pelvic floor and vagina with grafts or myocutaneous flaps.Uterine CancerEndometrial Cancer. Endometrial cancer is the most com-mon gynecologic malignancy and fourth most common cancer in women.96 It is most common in menopausal women in the fifth decade of life; up to 15% to 25% of cases occur prior to menopause, and 1% to 5% occur before age 40. Risk factors for the most common type of endometrial cancer include increased exposure to estrogen without adequate opposition by progester-one, either endogenous (obesity, chronic anovulation) or exog-enous (hormone replacement). Additional risk factors include diabetes, Lynch II syndrome (hereditary nonpolyposis coli syn-drome), and prolonged use of tamoxifen. Tamoxifen is a mixed agonist/antagonist ligand for


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Surgery_Schwartz. replacement). Additional risk factors include diabetes, Lynch II syndrome (hereditary nonpolyposis coli syn-drome), and prolonged use of tamoxifen. Tamoxifen is a mixed agonist/antagonist ligand for the estrogen receptor. It is an ago-nistic in the uterus and an antagonistic to the breast and ovary. Protective factors for endometrial cancer include smoking and use of combination oral contraceptive pills. Adenocarcinomas are the most prevalent histologic type.Endometrial adenocarcinomas have historically been divided into type I and type II tumors with five classic histologic subtypes. Type I tumors are estrogen-dependent endometrioid Brunicardi_Ch41_p1783-p1826.indd 181318/02/19 4:35 PM 1814SPECIFIC CONSIDERATIONSPART IIFigure 41-22. Pelvic exenteration.histology and have a relatively favorable prognosis; they can be broken down further by presence or absence of microsatellite instability. Type II endometrial cancers are estrogen-independent, aggressive, and characterized by


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Surgery_Schwartz. favorable prognosis; they can be broken down further by presence or absence of microsatellite instability. Type II endometrial cancers are estrogen-independent, aggressive, and characterized by nonendometrioid, serous or clear cell, histology, or carcinosarcoma.102 Emerging data, however, suggest that the molecular features could provide reproducible subtypes that have the potential to guide and refine treatment. The most comprehensive molecular study of endometrial cancer to date has been The Cancer Genome Atlas, which included a combination of whole genome sequencing, exome sequencing, microsatellite instability assays, copy number analysis, and proteomics.103 Molecular information was used to classify 232 endometrial cancer patients into four groups: POLE ultramutated, MSI hypermutated, copy number low, and copy number high that correlated with progression-free survival.103 Two practical pared-down classification systems to identify four molecular subgroups with distinct prognostic


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Surgery_Schwartz. copy number low, and copy number high that correlated with progression-free survival.103 Two practical pared-down classification systems to identify four molecular subgroups with distinct prognostic outcomes have been described.104,105Postmenopausal bleeding is the most common presenta-tion of endometrial cancer and often permits early stage diag-nosis, resulting in a favorable prognosis. Abnormal bleeding should prompt endometrial evaluation and sampling, which is usually done with an office endometrial biopsy, though at times requires operative curettage or diagnostic hysteroscopy. Transvaginal ultrasonography (TVUS) often reveals a thickened endometrial stripe. An endometrial stripe measuring 5 mm or more in a postmenopausal patient with vaginal bleeding raises concern and should be followed by endometrial sampling; patients with stripe of 4 mm or less rarely have occult malig-nancy, and TVUS may thus be used to triage patients before invasive endometrial sampling. Even with a


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Surgery_Schwartz. followed by endometrial sampling; patients with stripe of 4 mm or less rarely have occult malig-nancy, and TVUS may thus be used to triage patients before invasive endometrial sampling. Even with a normal endometrial stripe, endometrial sampling should be performed for persistent postmenopausal bleeding. Uterine cancer is surgically staged and is graded based on the degree of histologic differentiation of the glandular components (Table 41-9).99 Grade is an important prognostic factor, independent of stage.Treatment is surgical, and most commonly involves hysterectomy, bilateral salpingo-oophorectomy, peritoneal cytology, and resection of any gross disease.87 Evidence supports equivalent oncologic outcomes with minimally invasive approaches.106 The inclusion and utility of lymphadenectomy remains an area of controversy. If a lymph node dissection is performed, it may be performed via laparotomy or laparoscopy. Generally, the bilateral pelvic and para-aortic lymph nodes are removed.


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Surgery_Schwartz. remains an area of controversy. If a lymph node dissection is performed, it may be performed via laparotomy or laparoscopy. Generally, the bilateral pelvic and para-aortic lymph nodes are removed. The pelvic node dissection includes: bilateral removal of nodal tissue from the distal one-half of each common iliac artery, the anterior and medial aspect of the proximal half of the external iliac artery and vein, and the distal half of the obturator fat pad anterior to the obturator nerve. Most of the pelvic lymph nodes lie anterior, medially, and posteriorly to the external and internal iliac vessels and the obturator nerve. There are a few nodes that lie lateral to these structures, between the vessels and the pelvic sidewall, and these are generally removed in a complete dissection. The para-aortic lymph nodes include resection of nodal tissue over the distal vena cava from the level of the inferior mesenteric artery to the mid right common iliac artery and between the aorta and


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Surgery_Schwartz. The para-aortic lymph nodes include resection of nodal tissue over the distal vena cava from the level of the inferior mesenteric artery to the mid right common iliac artery and between the aorta and the left ureter from the inferior mesenteric artery to the left mid common iliac artery. Some also advocate resection of lymph nodes between the IMA and the gonadal vessels, as some uterine fundal tumors may drain directly into these lymph nodes.107The need for postoperative intervention is individualized based on the histology, stage, and risk factors such as age, lym-phvascular space invasion, and histology. Early-stage patients Table 41-92009 International Federation of Gynecology and Obstetrics staging of carcinoma of the uterine corpusI ATumor confined to the uterus, no or <½ myometrial invasionI BTumor confined to the uterus, >½ myometrial invasionIICervical stromal invasion, but not beyond uterusIII ATumor invades serosa or adnexaIII BVaginal and/or parametrial involvementIII


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Surgery_Schwartz. invasionI BTumor confined to the uterus, >½ myometrial invasionIICervical stromal invasion, but not beyond uterusIII ATumor invades serosa or adnexaIII BVaginal and/or parametrial involvementIII C1Pelvic-node involvementIII C2Para-aortic involvementIV ATumor invasion bladder and/or bowel mucosaIV BDistant metastases including abdominal metastases and/or inguinal lymph nodesData from Pecorelli S: Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium, Int J Gynaecol Obstet. 2009 May;105(2):103-104.Brunicardi_Ch41_p1783-p1826.indd 181418/02/19 4:35 PM 1815GYNECOLOGYCHAPTER 41are typically cured with surgery alone, while patients with high-intermediate risk factors, as defined by collaborative tri-als groups, commonly receive intracavitary brachytherapy to decrease local recurrence.108,109 Patients with advanced disease and high-grade histologies commonly receive platinum-based chemotherapy with or without radiation.Similar to the case with vulvar cancer described


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Surgery_Schwartz. recurrence.108,109 Patients with advanced disease and high-grade histologies commonly receive platinum-based chemotherapy with or without radiation.Similar to the case with vulvar cancer described earlier, sentinel node biopsy is becoming more prevalent in endome-trial cancer. A sentinel lymph node biopsy may be considered in apparent uterine-confined malignancy when there is no metasta-sis demonstrated by imaging studies or no obvious extrauterine disease at exploration. For this procedure, most frequently the cervix is injected with ICG dye, and the immunofluorescence detecting camera is used either robotically or laparoscopically to identify the sentinel node. If no node is mapped, a full lymph-adenectomy is generally advised.110Lynch Syndrome. Lynch syndrome, a cancer family syn-drome also known as hereditary nonpolyposis colorectal cancer (HNPCC), is an autosomal dominant inherited predisposition to develop colorectal carcinoma and extracolonic cancers, pre-dominantly including


Surgery_Schwartz

Surgery_Schwartz. also known as hereditary nonpolyposis colorectal cancer (HNPCC), is an autosomal dominant inherited predisposition to develop colorectal carcinoma and extracolonic cancers, pre-dominantly including tumors of the uterus and ovaries, now also including breast cancer.111 Genes involved in HNPCC are those required for proper single-strand DNA repair via the mismatch repair pathway; most commonly involved are MLH1, MSH2, MSH6, and PMS2. The risk of colorectal carcinoma is as high as 75% by age 75 years. Affected women have a 40% and 10% lifetime risk of developing uterine and ovarian cancers, respec-tively. Surveillance has not been proven to identify disease in early stage for these patients, though it is recommended and should include annual cervical cytology, mammography, trans-vaginal ultrasonography, CA-125 measurements, and an endo-metrial biopsy. Risk-reducing salpingo-oophorectomy with hysterectomy is now being recommended for women who have completed childbearing, ideally 5 to 10


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Surgery_Schwartz. CA-125 measurements, and an endo-metrial biopsy. Risk-reducing salpingo-oophorectomy with hysterectomy is now being recommended for women who have completed childbearing, ideally 5 to 10 years earlier than the first case of endometrial or ovarian cancer in the family. Dys-regulation of the mismatch repair pathway leads to the micro-satellite instability phenotype, now known be associated with susceptibility to select immunotherapy agents.Uterine Sarcomas. Uterine sarcomas arise from the uterine muscle and connective tissue elements and are typically aggres-sive tumors with a poorer prognosis compared to the more common endometrial carcinomas. The most common histopath-ologic types are endometrial stromal sarcomas, undifferentiated endometrial sarcomas, and leiomyosarcomas. Risk factors are challenging to assess but may include prior pelvic radiation and tamoxifen exposure. Patients typically present with bleeding or mass effects, although some are discovered incidentally at the time


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Surgery_Schwartz. challenging to assess but may include prior pelvic radiation and tamoxifen exposure. Patients typically present with bleeding or mass effects, although some are discovered incidentally at the time of hysterectomy for other indications. Leiomyosarcoma is the most common uterine sarcoma, and hysterectomy with salpingoophorectomy is the treatment of choice. Lymph node metastases are rare in sarcomas in general, and in the absence of palpable nodes or extrauterine disease. There are limited data to support cytoreduction when extrauterine disease is present. The benefits of adjuvant therapy are unknown. Advanced disease is typically treated with systemic chemotherapy.112Ovarian CancerEpithelial Ovarian, Tubal, and Primary Peritoneal Cancer. Ovarian cancer is a rare disease affecting 1 in 70 women with a median age at diagnosis of 62 years.96 Epithelial malignancies make up the vast majority of ovarian cancers. The majority of women (70%) are diagnosed at with advanced staged disease


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Surgery_Schwartz. 70 women with a median age at diagnosis of 62 years.96 Epithelial malignancies make up the vast majority of ovarian cancers. The majority of women (70%) are diagnosed at with advanced staged disease leading to the poor survival associated with this malignancy. Survival in advanced disease is due both to late diagnosis and lack of effective second-line cytotoxic therapy for the major-ity of patients who relapse following initial clinical complete response to platinum-based chemotherapy. Despite multiple pro-spective population based trials evaluating the use of CA-125, ultrasound, or combinations of these tests for early detection of disease, a mortality benefit to screening programs has not been demonstrated.113-116 Symptoms for either benign or malignant ovarian tumors are nonspecific but frequent, and they include bloating, pelvic or abdominal pain, difficulty eating or feeling full quickly, and urinary symptoms of urgency or frequency,117 which form the basis of an ovarian cancer


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Surgery_Schwartz. frequent, and they include bloating, pelvic or abdominal pain, difficulty eating or feeling full quickly, and urinary symptoms of urgency or frequency,117 which form the basis of an ovarian cancer symptom index (Table 41-10). When newly developed and persistent, these symptoms should prompt an evaluation specifically targeted for identification of gynecologic malignancy.The histologic heterogeneity of ovarian cancer has long been recognized, but with the emergence of more robust clini-copathologic, molecular, and genetic data over the past decade these distinctions have become more clearly defined. Type I tumors consist of low-grade serous (LGS), low-grade endome-trioid, clear cell carcinomas (CCC), and mucinous carcinomas and are characterized by mutations in KRAS, BRAF, PTEN, PIK3CA, CTNNB1, ARID1A, and PPP2R1A. Type II ovarian cancers are the most common of the ovarian cancer histotypes, consisting of high-grade serous (70%), high-grade endometri-oid, carcinosarcoma, and


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Surgery_Schwartz. PIK3CA, CTNNB1, ARID1A, and PPP2R1A. Type II ovarian cancers are the most common of the ovarian cancer histotypes, consisting of high-grade serous (70%), high-grade endometri-oid, carcinosarcoma, and undifferentiated carcinomas. Type II tumors are defined by TP53 mutations, which are rare in type I cancers.118-121 Each of these types have distinct risk factors and potential precursor lesions.121Risk factors for development of ovarian cancer include hormonal factors such as early menarche, late menopause, and nulliparity. The use of oral contraceptives reduces risk of ovar-ian carcinoma—this risk reduction persists for up to 30 years after cessation of use.122 Additionally, tubal ligation and hyster-ectomy decrease population level epithelial ovarian cancer risk. Genetic predisposition to breast or ovarian cancer is the most important known risk for the development of ovarian cancer, and 18% to 24% of ovarian carcinomas may arise in conjunction with a hereditary predisposition.123-128


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Surgery_Schwartz. breast or ovarian cancer is the most important known risk for the development of ovarian cancer, and 18% to 24% of ovarian carcinomas may arise in conjunction with a hereditary predisposition.123-128 Germline genetic muta-tions are far more common among type II ovarian cancers, while endometriosis and hormonal factors predispose to type I ovarian malignancies.121,126,129Since 2007, the National Comprehensive Cancer Network guidelines began recommending that all women diagnosed with ovarian cancer receive genetic testing as up to 20% of ovarian cancer patients are BRCA1/2 mutation carriers.127,130-134 Although family history of breast and/or epithelial ovarian cancer is one of the strongest factors for lifetime risk of having breast or epithelial ovarian cancer, up to 50% of women with ovarian cancer who test positive for a BRCA mutation have no fam-ily history of either malignancy, supporting the importance of testing all women with a personal diagnosis of ovarian cancer, regardless


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Surgery_Schwartz. cancer who test positive for a BRCA mutation have no fam-ily history of either malignancy, supporting the importance of testing all women with a personal diagnosis of ovarian cancer, regardless of family history. The identification of deleterious mutations allows for cascade testing. Relatives of the affected patient are referred for genetic testing limited to the identified mutation. The lifetime risk for the development of ovarian can-cer for carriers of mutations in the BRCA1 and BRCA2 genes Brunicardi_Ch41_p1783-p1826.indd 181518/02/19 4:35 PM 1816SPECIFIC CONSIDERATIONSPART IIis estimated to be between 20% and 45% and 10% and 20%, respectively.123,130,135One of the challenges associated with early detection of ovarian cancer has historically been the lack of an identifiable precursor lesion. In 2001, however, “dysplastic changes” in the fallopian tubes removed from women with increased risk of developing ovarian carcinoma were first described.136 Subse-quent careful


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Surgery_Schwartz. precursor lesion. In 2001, however, “dysplastic changes” in the fallopian tubes removed from women with increased risk of developing ovarian carcinoma were first described.136 Subse-quent careful microscopic examination using a newly developed “sectioning and extensively examining of the fimbriated end” protocol (SEE-FIM) of the grossly normal fallopian tubes and ovaries from women with BRCA1/2 mutations revealed occult tubal cancer and precancers designated as serous tubal intraepi-thelial carcinoma. The relationship between serous tubal intraep-ithelial carcinomas and high-grade serous and endometrioid cancers is supported by the ubiquitous presence of TP53 muta-tions and their typical location within the fimbriated end of the fallopian tube.118,121,137 High-grade, serous epithelial cancers of the ovary, fallopian tube, and peritoneum are now recognized to have a common fallopian tubal precursor lesion and often com-bined under the rubric of epithelial ovarian cancer (HGSOC).For


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Surgery_Schwartz. cancers of the ovary, fallopian tube, and peritoneum are now recognized to have a common fallopian tubal precursor lesion and often com-bined under the rubric of epithelial ovarian cancer (HGSOC).For women at increased risk of ovarian cancer, the only confirmed prevention strategy is risk-reducing salpingo-oopherectomy.138,139 The lifetime risk of HGSOC is reduced to under 3% with risk-reducing salpingo-oopherectomy. A modern understanding of the fallopian tube as the site of origin for many ovarian cancers has led to the suggestion that opportunistic salpingectomy could be implemented as a potential cancer prevention strategy in the general population. Scandinavian population-based cohort studies have demon-strated a significant decrease in epithelial ovarian cancer following salpingectomy.140,141 Opportunistic salpingectomy is feasible among women undergoing tubal ligation, hysterectomy, or other pelvic surgery.142 Early Staged Ovarian Cancer. Early stage epithelial ovarian cancer


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Surgery_Schwartz. Opportunistic salpingectomy is feasible among women undergoing tubal ligation, hysterectomy, or other pelvic surgery.142 Early Staged Ovarian Cancer. Early stage epithelial ovarian cancer has an excellent outcome. Low grade, stages IA and B disease can be cured in up to 90% to 95% of cases by a complete surgical procedure. The prevailing position in the United States is that such patients do not benefit from chemotherapy.143 8The standard of care for women with stages IC and II, and all women with grade 3 or clear cell histology, is adjuvant che-motherapy with 3 to 6 cycles of platinumand taxane-based chemotherapy.144Advanced Ovarian Cancer. A pelvic mass with ascites, an omental cake, and an elevated CA-125 is pathognomonic for advanced ovarian cancer. CT scan is the imaging modality of choice to evaluate the upper abdomen and potential resect-ability of disease. Concerning physical or radiographic exam findings should prompt referral to a gynecologic oncologist (Table 41-10), as


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Surgery_Schwartz. of choice to evaluate the upper abdomen and potential resect-ability of disease. Concerning physical or radiographic exam findings should prompt referral to a gynecologic oncologist (Table 41-10), as studies demonstrate inferior patient outcome for women who have had primary surgery by nongynecologic oncologists.The objectives of surgery in ovarian cancer are threefold. The first is to make the histologic diagnosis. The second is to assess the extent of disease through complete surgical staging (Tables 41-11 and 41-12). When epithelial ovarian cancer is identified on frozen section and disease is grossly limited to the pelvis, complete staging with node dissection will upstage nearly one-third of patients.145 The third objective is (when feasible) surgical cytoreduction or debulking. The extent of disease upon entering the abdomen and the residual disease upon completion of the debulking surgery are independent prognostic variables for patient outcome. The Gynecologic Oncology Group


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Surgery_Schwartz. extent of disease upon entering the abdomen and the residual disease upon completion of the debulking surgery are independent prognostic variables for patient outcome. The Gynecologic Oncology Group has defined optimal residual disease as residual tumor ≤1 cm in the largest diameter. However, more contemporary data suggest that the most favorable survival outcomes are associated with complete cytoreduction to no gross residual disease.146 Decisions about the benefits and risks of radical debulking for individual presentations and diverse pathology depend on the age and medical stability of the patient, as well as the pathologic type of the cancer.The publication of two randomized prospective trials of neoadjuvant chemotherapy (NACT) for ovarian cancer has led to a questioning of the dogma of maximum surgical effort. Both trials revealed no survival difference compared to primary deb-ulking.147,148 In a patient who is medically compromised or in whom complete primary cytoreduction is


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Surgery_Schwartz. of maximum surgical effort. Both trials revealed no survival difference compared to primary deb-ulking.147,148 In a patient who is medically compromised or in whom complete primary cytoreduction is unlikely, neoadjuvant Table 41-10Ovarian cancer symptom index (2007) and ACOG guidelines for patient referral to gynecologic oncologyOVARIAN CANCER SYMPTOM INDEXACOG GUIDELINES FOR REFERRAL OF PREMENOPAUSAL WOMEN WITH MASS SUSPICIOUS FOR OVCAACOG GUIDELINES FOR REFERRAL OF POSTMENOPAUSAL WOMEN WITH MASS SUSPICIOUS FOR OVCADevelopment of, change in, and/or persistence in:1 or more of:1 or more of:BloatingCA-125 >200 U/mLElevated CA-125Pelvic or abdominal painAscitesAscitesDifficulty eating or feeling full quicklyEvidence of abdominal or distant metastasisNodular or fixed pelvic massUrinary symptoms of urgency or frequencyFamily history of 1 or more first degree relatives with ovarian or breast cancerEvidence of abdominal or distant metastasisFamily history of one or more first-degree


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Surgery_Schwartz. symptoms of urgency or frequencyFamily history of 1 or more first degree relatives with ovarian or breast cancerEvidence of abdominal or distant metastasisFamily history of one or more first-degree relatives with ovarian or breast cancer ACOG = American Congress of Obstetricians and Gynecologists.Data from Goff BA, Mandel LS, Drescher CW, et al. Development of an ovarian cancer symptom index: possibilities for earlier detection. Cancer. 2007;109:221-227; Dearking AC, Aletti GD, McGree ME, Weaver AL, Sommerfield MK, Cliby WA. How relevant are ACOG and SGO guidelines for referral of adnexal mass? Obstet Gynecol. 2007;110:841-848.Brunicardi_Ch41_p1783-p1826.indd 181618/02/19 4:35 PM 1817GYNECOLOGYCHAPTER 41Table 41-112014 International Federation of Gynecology and Obstetrics staging of epithelial ovarian cancerITumor confined to ovaries or fallopian tube(s)T1IATumor limited to one ovary (capsule intact) or fallopian tubeNo tumor on ovarian or fallopian tube surfaceNo malignant


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Surgery_Schwartz. of epithelial ovarian cancerITumor confined to ovaries or fallopian tube(s)T1IATumor limited to one ovary (capsule intact) or fallopian tubeNo tumor on ovarian or fallopian tube surfaceNo malignant cells in the ascites or peritoneal washingsT1aIBTumor limited to both ovaries (capsules intact) or fallopian tubesNo tumor on ovarian or fallopian tube surfaceNo malignant cells in the ascites or peritoneal washingsT1bICTumor limited to one or both ovaries or fallopian tubes, with any of the following:IC1 Surgical spill intraoperativelyIC2 Capsule ruptured before surgery or tumor on ovarian or fallopian tube surfaceIC3 Malignant cells present in the ascites or peritoneal washingsT1cIITumor involves one or both ovaries or fallopian tubes with pelvic extension (below pelvic brim) or peritoneal cancer (Tp)T2IIAExtension and/or implants on the uterus and/or fallopian tubes/and/or ovariesT2aIIBExtension to other pelvic intraperitoneal tissuesT2bIIITumor involves one or both ovaries, or


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Surgery_Schwartz. cancer (Tp)T2IIAExtension and/or implants on the uterus and/or fallopian tubes/and/or ovariesT2aIIBExtension to other pelvic intraperitoneal tissuesT2bIIITumor involves one or both ovaries, or fallopian tubes, or primary peritoneal cancer, with cytologically or histologically confirmed spread to the peritoneum outside the pelvis and/or metastasis to the retroperitoneal lymph nodesT3IIIAMetastasis to the retroperitoneal lymph nodes with or without microscopic peritoneal involvement beyond the pelvisT1, T2, T3aN1IIIA1Positive retroperitoneal lymph nodes only (cytologically or histologically proven) IIIA1(i)Metastasis ≤10 mm in greatest dimension (note this is tumor dimension and not lymph node dimension)T3a/T3aN1IIIA1(ii)Metastasis >10 mm in greatest dimension IIIA 2Microscopic extrapelvic (above the pelvic brim) peritoneal involvement with or without positive retroperitoneal lymph nodesT3a/T3aN1IIIBMacroscopic peritoneal metastases beyond the pelvic brim ≤2 cm in greatest dimension,


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Surgery_Schwartz. (above the pelvic brim) peritoneal involvement with or without positive retroperitoneal lymph nodesT3a/T3aN1IIIBMacroscopic peritoneal metastases beyond the pelvic brim ≤2 cm in greatest dimension, with or without metastasis to the retroperitoneal lymph nodesT3b/T3bN1III CMacroscopic peritoneal metastases beyond the pelvic brim >2 cm in greatest dimension, with or without metastases to the retroperitoneal nodes (Note 1)T3c/T3cN1IVDistant metastasis excluding peritoneal metastases Stage IV A: Pleural effusion with positive cytologyStage IV B: Metastases to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside of abdominal cavity) (Note 2)Any T, any N, M1Reproduced with permission from Mutch DG, Prat J: 2014 FIGO staging for ovarian, fallopian tube and peritoneal cancer, Gynecol Oncol. 2014 Jun; 133(3):401-404.Table 41-12Components of comprehensive surgical staging and debulking of epithelial ovarian cancerVertical abdominal incision adequate to visualize the


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Surgery_Schwartz. cancer, Gynecol Oncol. 2014 Jun; 133(3):401-404.Table 41-12Components of comprehensive surgical staging and debulking of epithelial ovarian cancerVertical abdominal incision adequate to visualize the diaphragmsEvacuation of ascitesPeritoneal washings of each pelvic gutter and diaphragmEn bloc hysterectomy and bilateral salpingo-oopherectomyInfragastric omentectomyRetroperitoneal and pelvic lymph node dissectionExamination of the entire bowelRandom biopsies of apparently uninvolved areas of peritoneum, pericolic gutters, diaphragmchemotherapy followed by interval debulking may be more appropriate and is supported by recent randomized controlled trials. Typically, treatment with NACT includes three cycles of platinum-based chemotherapy prior to open debulking, then three additional cycles after surgery. Diagnostic laparoscopic evaluation prior to cytoreductive surgery has been suggested as a means to avoid unnecessary laparotomy, resulting in subop-timal cytoreduction. Patients deemed


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Surgery_Schwartz. after surgery. Diagnostic laparoscopic evaluation prior to cytoreductive surgery has been suggested as a means to avoid unnecessary laparotomy, resulting in subop-timal cytoreduction. Patients deemed not to be candidates for cytoreduction could proceed immediately to NACT at the time of tissue collection for definitive diagnosis. A Fagotti predictive index ≥8 (Table 41-13) is a predictor of suboptimal cytoreduc-tion in advanced ovarian cancer with reasonable sensitivity and high specificity.149 These recommendations currently apply to HGSOC, clear cell cancer, and high-grade endometrioid ovarian Brunicardi_Ch41_p1783-p1826.indd 181718/02/19 4:35 PM 1818SPECIFIC CONSIDERATIONSPART IIcancers. Low-grade tumors are less chemotherapy sensitive, and primary surgical resection is recommended when feasible. Standard of care adjuvant therapy of advanced stage epithe-lial ovarian cancer remains intravenous platinumand tax-ane-based chemotherapy.150 In 2006, the National Cancer Institute


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Surgery_Schwartz. when feasible. Standard of care adjuvant therapy of advanced stage epithe-lial ovarian cancer remains intravenous platinumand tax-ane-based chemotherapy.150 In 2006, the National Cancer Institute issued a clinical alert indicating that combination intrave-nous/intraperitoneal platinum/taxane postoperative chemotherapy should be considered first line for women with optimally cytore-duced EOC. This was the result of completion and analysis of three independent randomized clinical trials showing a significant survival advantage for intraperitoneal therapy.151,152 Intraperitoneal (IP) therapy is administered via an implanted 9.6 French venous port catheter with the port placed over the right or left costal 9margin. The catheter is tunneled caudad with insertion through the fascia in the lower abdomen and the tip in the pelvis. The IP cath-eter may be placed at the time of surgical debulking via an open laparotomy approach or prior to initiating chemotherapy via a laparoscopic approach. In


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Surgery_Schwartz. abdomen and the tip in the pelvis. The IP cath-eter may be placed at the time of surgical debulking via an open laparotomy approach or prior to initiating chemotherapy via a laparoscopic approach. In some centers, the IP catheter may be placed by interventional radiology with CT guidance.Patients who have suboptimally debulked advanced stage disease and/or who are not candidates for intraperitoneal ther-apy should receive intravenous adjuvant chemotherapy. Interest has increased in both dose dense IV chemotherapy dosing as well as incorporation of biologic agents.Secondary cytoreduction upon recurrence can be con-sidered (Table 41-14). Patients who have had a disease-free Table 41-13Laparoscopic assessment of advanced ovarian cancer to predict surgical resectabilityLAPAROSCOPIC FEATURESCORE 0SCORE 2Peritoneal carcinomatosisCarcinomatosis involving a limited area (along the paracolic gutter or the pelvic peritoneum) and surgically removable by peritonectomyUnresectable massive


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Surgery_Schwartz. 0SCORE 2Peritoneal carcinomatosisCarcinomatosis involving a limited area (along the paracolic gutter or the pelvic peritoneum) and surgically removable by peritonectomyUnresectable massive peritoneal involvement as well as with a miliary pattern of distributionDiaphragmatic diseaseNo infiltrating carcinomatosis and no nodules confluent with the most part of the diaphragmatic surfaceWidespread infiltrating carcinomatosis or nodules confluent with the most part of the diaphragmatic surfaceMesenteric diseaseNo large infiltrating nodules and no involvement of the root of the mesentery as would be indicated by limited movement of the various intestinal segmentsLarge infiltrating nodules or involvement of the root of the mesentery indicated by limited movement of the various intestinal segmentsOmental diseaseNo tumor diffusion observed along the omentum up to the large stomach curvatureTumor diffusion observed along the omentum up to the large stomach curvatureBowel infiltrationNo bowel


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Surgery_Schwartz. diseaseNo tumor diffusion observed along the omentum up to the large stomach curvatureTumor diffusion observed along the omentum up to the large stomach curvatureBowel infiltrationNo bowel resection was assumed and no miliary carcinomatosis on the ansae observedBowel resection assumed or miliary carcinomatosis on the ansae observedStomach infiltrationNo obvious neoplastic involvement of the gastric wallObvious neoplastic involvement of the gastric wallLiver metastasesNo surface lesionsAny surface lesionTable 41-14Guidelines for secondary therapy of epithelial ovarian cancerTIME FROM COMPLETION OF PRIMARY THERAPYDEFINITIONINTERVENTIONProgression on therapyPlatinum-refractoryNo value of secondary debulking unless remediating complication such as bowel obstructionNon–platinum-based chemotherapyConsider clinical trialProgression within 6 months of completion of primary therapyPlatinum-resistantNo value of secondary debulking unless remediating complication such as bowel


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Surgery_Schwartz. chemotherapyConsider clinical trialProgression within 6 months of completion of primary therapyPlatinum-resistantNo value of secondary debulking unless remediating complication such as bowel obstructionNon–platinum-based chemotherapy consider adding bevacizumabConsider clinical trialProgression after 6 months post completion of primary therapyPlatinum-sensitiveConsider secondary debulking if greater than 12 months intervalConsider platinum +/− taxane +/− bevacizumab, +/− pegylated liposomal doxorubicin, +/− gemcitabineConsider maintenance PARP inhibitorConsider clinical trialBrunicardi_Ch41_p1783-p1826.indd 181818/02/19 4:35 PM 1819GYNECOLOGYCHAPTER 41period of at least 12 months following an initial complete clini-cal response to surgery and initial chemotherapy, who have no evidence of carcinomatosis on imaging, and who have disease that can be completely resected are considered optimal candi-dates. A randomized controlled trial reported in abstract form demonstrated a benefit


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Surgery_Schwartz. of carcinomatosis on imaging, and who have disease that can be completely resected are considered optimal candi-dates. A randomized controlled trial reported in abstract form demonstrated a benefit of secondary cytoreduction under strict entry criteria (DESKTOP3); the GOG-0213 study of secondary cytoreduction is maturing. Debulking surgery done after subse-quent relapses or in women with early recurrence has not been shown to result in an outcome benefit and should be used only to palliate disease complications.The most common cause of palliative surgery is bypass of bowel obstruction. The majority of women with advanced ovarian cancer will eventually develop and potentially die from malignant bowel obstruction. While management of these cases is controversial, in some cases surgical correction has been shown to prolong life and improve quality of life.153 Nonsurgical options include placement of a venting gastrostomy tube, per-formed endoscopically or surgically. Management of


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Surgery_Schwartz. correction has been shown to prolong life and improve quality of life.153 Nonsurgical options include placement of a venting gastrostomy tube, per-formed endoscopically or surgically. Management of malignant bowel obstruction in women with recurrent advanced disease should be individualized.Chemotherapy is the mainstay of therapy for recurrent EOC. Treatment approaches are based upon platinum sensitivity.154 Referral to an oncologist with specific expertise in chemothera-peutic treatment of ovarian cancer and access to clinical trials is important. In determining secondary and subsequent ther-apy, consideration of prior therapies, sites of disease, organs at risk from cancer, organs sustaining injury from prior ther-apy, and quality of life desires of patient should be taken into consideration.Ovarian Germ Cell Tumors. Ovarian germ cell tumors occur most commonly in women under age 30. The most common benign germ cell neoplasm is the mature cystic teratoma; approximately 1% of


Surgery_Schwartz

Surgery_Schwartz. Germ Cell Tumors. Ovarian germ cell tumors occur most commonly in women under age 30. The most common benign germ cell neoplasm is the mature cystic teratoma; approximately 1% of teratomas contain a secondary malig-nancy arising from one of the components, most commonly squamous cell cancer and most commonly in postmenopausal women. Malignant germ cell tumors often grow and dissemi-nate rapidly and are symptomatic. The rapid growth may be accompanied by torsion or rupture, producing an acute abdo-men and the need for emergent intervention. Because they are derived from primordial germ cells, many produce charac-teristic tumor markers. Immature teratomas comprise a sig-nificant proportion of malignant germ cell tumors and may be associated with elevated lactate dehydrogenase (LDH) or α-fetoprotein (AFP). Excluding teratomas, the most common malignant germ cell tumor is dysgerminoma, made up of pure undifferentiated germ cells. Bilaterality occurs in up to 15% of patients; lactate


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Surgery_Schwartz. α-fetoprotein (AFP). Excluding teratomas, the most common malignant germ cell tumor is dysgerminoma, made up of pure undifferentiated germ cells. Bilaterality occurs in up to 15% of patients; lactate dehydrogenase is commonly elevated, and elevated b-hCG may occur.Less common malignant germ cell tumors include endo-dermal sinus or yolk sac tumors, embyronal carcinomas, mixed germ cell neoplasms, polyembryomas, and choriocarcinomas. Endodermal sinus tumors may have elevated AFP levels in the blood while embryonal and mixed germ cell tumors may have elevated b-hCG, LDH, or AFP. Tumor markers are useful to fol-low during surveillance and definitive therapy. Other than com-pletely resected stage I, grade I immature teratoma, adjuvant chemotherapy with a platinum-containing regimen has been his-torically recommended.155 Because of the high response rates to chemotherapy and the long-term toxicity of treatment, a “watch and wait” approach with treatment only upon recurrence has been


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Surgery_Schwartz. been his-torically recommended.155 Because of the high response rates to chemotherapy and the long-term toxicity of treatment, a “watch and wait” approach with treatment only upon recurrence has been suggested as safe for selected, well-staged patients with germ cell tumors.156 The cure rate remains high, near 90% even when metastatic disease is present; recurrent disease is more difficult to eradicate.155Fertility preservation is the standard surgical approach for ovarian germ cell tumors as disease tends to be diagnosed at stage I, and salvage chemotherapy is overall extremely suc-cessful. Staging should include removal of the involved ovary, biopsy of any suspicious areas, pelvic and para-aortic node dis-section, and omentectomy. Hysterectomy or removal of the sec-ond ovary is rarely indicated.Growing teratoma syndrome is a rare sequela of germ cell malignancies. Characteristically, during or after chemotherapy slow-growing tumors will increase in size and may even com-press


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Surgery_Schwartz. indicated.Growing teratoma syndrome is a rare sequela of germ cell malignancies. Characteristically, during or after chemotherapy slow-growing tumors will increase in size and may even com-press surrounding organs. Malignant transformation within these masses has been described. Treatment is with surgical resection.157Ovarian Sex Cord-Stromal Tumors. Sex cord-stromal cell tumors, rare tumors, are derived from cells that support and surround the oocyte and can present with symptoms referable to endocrine activity of the tumor. These include granulosa cell tumors (female differentiated), fibroma-thecomas, and Sertoli-Leydig cell tumors (male differentiated). Granulosa cell tumors are the most common in this group and are a low-grade malignancy with fewer than 3% bilaterality. They are treated with conservative surgery, similar to germ cell tumors in young women.155 Hysterectomy and bilateral salpingo-oophorectomy is recommended for women who have completed childbearing. Nodal staging


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Surgery_Schwartz. with conservative surgery, similar to germ cell tumors in young women.155 Hysterectomy and bilateral salpingo-oophorectomy is recommended for women who have completed childbearing. Nodal staging can be safely omitted in the absence of grossly involve nodes and fertility preservation is possible in disease limited to one ovary, the most common presentation. Debulking surgery is recommended for more extensive disease. These tumors and the thecomas in the same class often stimulate estrogen production and can be found in association with endometrial hyperplasia and cancer (5%). Granulosa cell tumors can recur over a prolonged period given their low rate of proliferation and tendency for local or intraperitoneal recurrence. Inhibin has been shown to be elaborated by these tumors and often is followed to identify recurrence of the disease. The Sertoli/Leydig cell tumors can present with virilization as a primary symptom. Evaluation of the ovary when this symptom is found is always of


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Surgery_Schwartz. is followed to identify recurrence of the disease. The Sertoli/Leydig cell tumors can present with virilization as a primary symptom. Evaluation of the ovary when this symptom is found is always of value.Gestational Trophoblastic Disease. Gestational trophoblas-tic disease (GTD) is a spectrum of abnormal pregnancy–related trophoblastic proliferations. Premalignant histologic types include partial and complete hydatidiform moles. Primary sur-gery for diagnosis and initial therapy is a suction dilatation and curettage. Clinically, partial moles present as missed abortions and usually resolve with observation. Partial moles are triploid, usually XXY, which can result from dispermic fertilization of an egg. A previously described classical presentation of hyper-emesis gravidarum, hyperthyroidism, preeclampsia, pulmonary trophoblastic embolization, and uterine size larger than dates is rarely seen today because of routine ultrasound assessments during early pregnancy. Even in the first


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Surgery_Schwartz. preeclampsia, pulmonary trophoblastic embolization, and uterine size larger than dates is rarely seen today because of routine ultrasound assessments during early pregnancy. Even in the first trimester, however, a characteristic “snow storm” appearance may be seen on ultra-sound. Pathologic examination will demonstrate no fetal tissue and have a diploid karyotype resulting from paternal duplication occurring after loss of maternal genetic material, or occasionally Brunicardi_Ch41_p1783-p1826.indd 181918/02/19 4:35 PM 1820SPECIFIC CONSIDERATIONSPART IIwith dispermic fertilization of an empty egg. Often associated theca lutein ovarian cysts, which can be greater than 6 cm in diameter, are seen on ultrasound. They should be followed without surgical intervention as they resolve with removal or treatment of the GTD. Following uterine evacuation, patients with molar pregnancies must be followed closely with weekly b-hCGs until normal for 3 weeks and then monthly for at least 6 months.


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Surgery_Schwartz. or treatment of the GTD. Following uterine evacuation, patients with molar pregnancies must be followed closely with weekly b-hCGs until normal for 3 weeks and then monthly for at least 6 months. Contraception should be provided to allow for sur-veillance. Any increase in b-hCG should trigger further evalua-tion and consideration of chemotherapy.158,159Invasive moles, choriocarcinoma, and placental site tro-phoblastic tumors are malignant disorders. Invasive moles are diagnosed following the diagnosis of a molar pregnancy if any of the following are demonstrated: (a) a plateau of b-hCG lasts for four measurements over a period of 3 weeks or longer; (b) a rise in b-hCG for three consecutive weekly measurements over at least a period of 2 weeks or more; or (c) b-hCG level remains elevated for 6 months or more. Metastatic GTD can present on the cervix, vagina, liver, lung, or brain and should not be man-aged surgically. In a woman of reproductive age, a diagnosis of metastatic GTN can be


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Surgery_Schwartz. 6 months or more. Metastatic GTD can present on the cervix, vagina, liver, lung, or brain and should not be man-aged surgically. In a woman of reproductive age, a diagnosis of metastatic GTN can be made without biopsy if a b-hCG is found to be elevated in the setting of widespread metastatic disease. In fact, given the incidence of bleeding complications biopsy is not recommend.Chemotherapy is the primary recommended therapy. Per 2000 FIGO staging and classification, a risk score of 6 and below is classified as low risk and above 6 is considered high risk (Table 41-15). Low-risk patients are treated with single agent chemotherapy (methotrexate or actinomycin-D); high-risk patients receive multiagent chemotherapy. In either case, chemotherapy continues until b-hCG levels have normalized. Modern salvage and cure rates are high, with 5-year survival of high-risk patients reported as high as 90%.160 Twelve months of surveillance with contraception is recommended following treatment in


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Surgery_Schwartz. Modern salvage and cure rates are high, with 5-year survival of high-risk patients reported as high as 90%.160 Twelve months of surveillance with contraception is recommended following treatment in order to allow complete surveillance for relapse.Beyond dilation and curettage, surgery may have a role in the management of GTD. Hysterectomy is recommended for placental site trophoblastic tumors for which metastasis is rare. Laparotomy may be indicated in the cases of uncontrolled intra-abdominal or uterine bleeding. Neurosurgery may be required if there is intracranial bleeding or increased intracranial pressure due to metastatic disease.159MINIMALLY INVASIVE GYNECOLOGIC SURGERYHysteroscopySee earlier section, “Hysteroscopy” under “Procedures Per-formed for Structural Causes of Abnormal Uterine Bleeding.”LaparoscopyThe standard method for gynecologic laparoscopy follows the same methods as all minimally invasive surgery. In general, a camera port is placed near the umbilicus. Sometimes


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Surgery_Schwartz. Bleeding.”LaparoscopyThe standard method for gynecologic laparoscopy follows the same methods as all minimally invasive surgery. In general, a camera port is placed near the umbilicus. Sometimes it must be placed more cephalad if the patient has a larger fibroid uterus. Two additional ports are placed laterally, usually just superior and medial to the anterior superior iliac spines. Single site lapa-roscopic procedures may improve cosmesis and reduce post-operative pain, but challenges including lack of triangulation and instrument crowding at the umbilicus make this technique challenging to apply to more complex procedures.161Robotic SurgeryOver the last decade, there has been increased use of robot-ics for gynecologic surgery. With the DaVinci robotic system, the surgeon sits at a console and visualizes the operative field with three-dimensional optics. The use of robotic surgery has been described for virtually every gynecologic procedure that has been performed abdominally or


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Surgery_Schwartz. and visualizes the operative field with three-dimensional optics. The use of robotic surgery has been described for virtually every gynecologic procedure that has been performed abdominally or laparoscopically. The lapa-roscopic instruments are “wristed” and move as the surgeon’s hands/fingers move the actuators at the console. Robotic surgery Table 41-15International Federation of Gynecology and Obstetrics/World Health Organization scoring system for gestational trophoblastic disease based on prognostic factors SCORE 0124Age<40>40––Antecedent pregnancyMoleAbortionTermInterval from index pregnancy, months<44–67–12>12Pretreatment hCG mIU/mL<103>103–104>104–105>105Largest tumor size including uterus, cm–3–4≥5–Site of metastases including uterusLungSpleen, kidneyGastrointestinal tractBrain, liverNumber of metastases identified–1–45–8>8Previous failed chemotherapy––Single drugTwo or more drugsBrunicardi_Ch41_p1783-p1826.indd 182018/02/19 4:35 PM 1821GYNECOLOGYCHAPTER 41uses a camera


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Surgery_Schwartz. liverNumber of metastases identified–1–45–8>8Previous failed chemotherapy––Single drugTwo or more drugsBrunicardi_Ch41_p1783-p1826.indd 182018/02/19 4:35 PM 1821GYNECOLOGYCHAPTER 41uses a camera port, two to three robotic ports, and an accessory port. More meticulous dissection, improved visualization, and ability to operate with lower intra-abdominal pressures make the robotic platform advantageous, especially in obese patients. Longer set-up time and increased cost, however, are distinct disadvantages. The robotic unit costs up to $2.3 million and is associated with annual maintenance costs of $180,000 a year.162There is significant data to support robotic surgery in gynecologic malignancy; however, most procedures can be per-formed successfully with either robotic or laparoscopic platform depending on operator comfort and skill set. One large study sug-gested a lower conversion to laparotomy rate for robotic versus laparoscopic hysterectomy, but this was not statistically


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Surgery_Schwartz. platform depending on operator comfort and skill set. One large study sug-gested a lower conversion to laparotomy rate for robotic versus laparoscopic hysterectomy, but this was not statistically signifi-cant: conversion to laparotomy for laparoscopic hysterectomy was 9.9% compared with 4.9% for robotic cases (P =.06).163Complications Pertinent to Gynecologic SurgeryAbdominal Wall Vessels. The vessel at greatest risk of injury during the lateral trocar placement is the inferior epigastric artery. The superficial epigastric vessels and the superficial circumflex iliac vessels can be injured as well (Fig. 41-23). The primary methods to avoid vessel injury are knowledge of the vessels at risk and their visualization prior to trocar placement, when possible. The superficial vessels often can be seen and avoided by transillumination of the abdominal wall with the laparoscope. In contrast, the larger inferior epigastric vessels cannot be seen by transillumination because of their deeper


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Surgery_Schwartz. be seen and avoided by transillumination of the abdominal wall with the laparoscope. In contrast, the larger inferior epigastric vessels cannot be seen by transillumination because of their deeper location; these vessels often can be seen laparoscopically and avoided as they course along the peritoneum between the lateral umbilical fold of the bladder and the insertion of the round ligament into the inguinal canal. Anatomic variation and anastomoses between vessels make it impossible to know the exact location of all the abdominal wall vessels. For this reason, other strategies also should be used to avoid vessel injury, including the use of trocars with conical tips rather than pyramid tips and the use of the smallest trocars possible lateral to the midline.Intestinal Injury. Another potentially serious complication of laparoscopic surgery is injury to either small or large intestines. 10An estimated incidence of bowel injury during laparoscopic gynecologic surgery is estimated to be


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Surgery_Schwartz. serious complication of laparoscopic surgery is injury to either small or large intestines. 10An estimated incidence of bowel injury during laparoscopic gynecologic surgery is estimated to be 0.13%, 41% of which had a delayed diagnosis.164 Bowel injury can occur at the time of trocar insertion, especially if the patient has had previous abdominal procedures that often result in bowel adhesions to the anterior abdominal wall peritoneum, but rates appear simi-lar regardless of entry technique. Due to the proximity of sur-gery to the bowel, thermal injury due to electrosurgery is also frequently implicated in intestinal injury. Time to diagnosis in these cases is typically several days postoperatively as a thermal injury takes time to mature and necrose.Urologic Injuries. A risk of injury to the urogenital tract is inherent to gynecologic surgery due to proximity. Prevention of injury and intraoperative recognition and repair are crucial to avoiding long-term sequelae. Most urogenital


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Surgery_Schwartz. to the urogenital tract is inherent to gynecologic surgery due to proximity. Prevention of injury and intraoperative recognition and repair are crucial to avoiding long-term sequelae. Most urogenital fistulae are the result of unrecognized injuries to the urogenital tract at the time of surgery.Bladder Injury. Placement of a Foley catheter prior to gyne-cologic surgery is critical to reducing risk of bladder injuries. Bladder injury during open or laparoscopic surgery results from retroperitoneal perforation during lower trocar placement or during sharp dissection of the bladder from the lower uterine segment during hysterectomy. The latter of these two situa-tions is usually recognized intraoperatively; the first sign of the former may be postoperative hematuria, lower-port incisional drainage, or pneumoturia during laparoscopy. Once diagnosed, large defects require layered closure, whereas smaller defects usually close spontaneously within days or weeks with the aid of transurethral


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Surgery_Schwartz. or pneumoturia during laparoscopy. Once diagnosed, large defects require layered closure, whereas smaller defects usually close spontaneously within days or weeks with the aid of transurethral catheter drainage.Ureteral Injury. Although ureteral injury is rare, occurring in less than 1% of gynecologic procedures, it is the most serious of the complications related to gynecologic surgery, particularly if unrecognized.165,166 There are three anatomic locations where the ureter is at risk during gynecologic procedures (see Fig. 41-5): (a) the ureter descends over the pelvic brim as it courses over the bifurcation of the common iliac artery into the external and internal iliac arteries just below the ovarian vessels; (b) in the pelvis, the ureter courses along the lateral aspect of the broad ligament to enter the base of the broad ligament; and (c) the ure-ter is found less than 2 cm lateral to the cervix, passing under the uterine artery and then medially over the anterior vaginal


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Surgery_Schwartz. broad ligament to enter the base of the broad ligament; and (c) the ure-ter is found less than 2 cm lateral to the cervix, passing under the uterine artery and then medially over the anterior vaginal for-nix before entering the trigone of the bladder—this is the most common location of ureteral injury. Ureteral injuries, including complete ligation, partial resection, or thermal injuries, usually will manifest within hours to days of surgery. Complete obstruc-tion most often manifests as flank pain, whereas the first sign of partial or complete transection may be symptoms of intra-abdominal irritation caused by urine leakage. Transperitoneal thermal injuries resulting from fulguration of endometriosis may be similar to those after transection, but the appearance of symp-toms may be delayed several days until tissue necrosis occurs.Routine cystoscopy following hysterectomy is advocated by some gynecologists. For procedures performed for prolapse or incontinence where injury to the


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Surgery_Schwartz. several days until tissue necrosis occurs.Routine cystoscopy following hysterectomy is advocated by some gynecologists. For procedures performed for prolapse or incontinence where injury to the urinary tract is highest, rou-tine cystoscopy is recommended. Consideration of a surgeon’s individual complication rate and the difficulty of an individ-ual procedure are considerations for the provision of routine cystoscopy.166Vaginal Vault Dehiscence. This complication of hysterec-tomy seems to be more common in laparoscopic and robotic DeepvesselsSuperficial vessels Inferiorepigastric DeepcircumflexiliacSuperficial epigastricSuperficialcircumflex iliacFigure 41-23. Location of anterior abdominal wall blood vessels.Brunicardi_Ch41_p1783-p1826.indd 182118/02/19 4:35 PM 1822SPECIFIC CONSIDERATIONSPART IIsurgeries. This may be due to the use of cautery in dividing the vaginal cuff or in the method of vaginal closure when done mini-mally invasively. Vaginal closure of the cuff


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Surgery_Schwartz. CONSIDERATIONSPART IIsurgeries. This may be due to the use of cautery in dividing the vaginal cuff or in the method of vaginal closure when done mini-mally invasively. Vaginal closure of the cuff appears to decrease the rate of vaginal cuff dehiscence in MIS hysterectomy.Hemodynamically stable women without bowel eviscera-tion may be candidates for transvaginal repair without abdomi-nal exploration. Vaginal approach may also be appropriate in select cases of evisceration in which the bowel can be com-pletely evaluated vaginally. Since bowel evisceration can lead to peritonitis and sepsis, all women with bowel eviscerations are considered to have a surgical emergency, and surgery should not be delayed for imaging. In most cases of bowel eviscera-tion, evaluation of the bowel by laparoscopy or laparotomy is indicated to ensure bowel integrity.REFERENCES 1. Anson B. Atlas of Human Anatomy. Philadelphia: WB Saunders, 1950. 2. Force USPST. Screening for gynecologic conditions with pel-vic


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Surgery_Schwartz. or laparotomy is indicated to ensure bowel integrity.REFERENCES 1. Anson B. Atlas of Human Anatomy. Philadelphia: WB Saunders, 1950. 2. Force USPST. Screening for gynecologic conditions with pel-vic examination: US Preventive Services Task Force recom-mendation statement. JAMA. 2017;317:947-953. 3. McNicholas C, Peipert JF. Is it time to abandon the routine pel-vic examination in asymptomatic nonpregnant women? JAMA. 2017;317:910-911. 4. Schiffman M, Wentzensen N, Wacholder S, Kinney W, Gage JC, Castle PE. Human papillomavirus testing in the prevention of cervical cancer. J Natl Cancer Inst. 2011;103:368-383. 5. US Preventive Services Task Force. Cervical cancer: screen-ing. Available at: https://www.uspreventiveservicestaskforce.org/Page/Document/RecommendationStatementFinal/cervi-cal-cancer-screening2. Accessed August 11, 2018. 6. US Preventive Services Task Force. Screening for Cervical CancerUS Preventive Services Task Force Recommendation Statement. JAMA. 2018;320(7):674-686.


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Surgery_Schwartz. Accessed August 11, 2018. 6. US Preventive Services Task Force. Screening for Cervical CancerUS Preventive Services Task Force Recommendation Statement. JAMA. 2018;320(7):674-686. 7. Petrosky E, Bocchini Jr JA, Hariri S, et al. Use of 9-valent human papillomavirus (HPV) vaccine: updated HPV vac-cination recommendations of the advisory committee on immunization practices. MMWR Morb Mortal Wkly Rep. 2015;64:300-304. 8. Paavonen J, Naud P, Salmeron J, et al. Efficacy of human papillomavirus (HPV)-16/18 AS04-adjuvanted vaccine against cervical infection and precancer caused by onco-genic HPV types (PATRICIA): final analysis of a double-blind, randomised study in young women. Lancet. 2009;374: 301-314. 9. Meites E. Use of a 2-dose schedule for human papillomavi-rus vaccination—updated recommendations of the Advisory Committee on Immunization Practices. MMWR Morb Mortal Wkly Rep. 2016;65(49):1405-1408. 10. Gynecologists ACoOa. ACOG committee opinion: number 280, December 2002. The role of


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