10.5826/dpc.1201a33
text/xml
2160-9381
1883
en
Giuseppe Argenziano
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Dermatofibrosarcoma Protuberans: Experience at a Third-Level Referral Center
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Commentary
2022-02-02
2021-07-14
2022-02-02
2022-02-09
e2022033
Dermatol Pract Concept
Giuseppe Argenziano
2022/02/02
10.5826/dpc.1201a33
e2022033
Dermatology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania Luigi Vanvitelli, Naples, Italy 
2160-9381
en
e2022033
Criscione VD, Weinstock MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol. 2007;56(6):968-973. DOI: 10.1016/j.jaad.2006.09.006. PMID: 17141362.
Dermatofibrosarcoma Protuberans: Experience at a Third-Level Referral Center
https://dpcj.org/index.php/dpc/article/download/1883/1499
Dermatofibrosarcoma Protuberans: Experience at a
           Third-Level Referral Center
          Stefano Caccavale1, Adriana Martins Basso2, Paola Vitiello1, Andrea Ronchi3,
        Antonello Sica4, Pasquale Verolino5, Ruzica Jurakic Toncic6, Giuseppe Argenziano1


1 Dermatology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania Luigi Vanvitelli,
  Naples, Italy
2 Medicina Integrada, private practice, Sao Paulo, Brasil
3 Pathology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania Luigi Vanvitelli, Naples, Italy
4 Oncology and Hematology Unit, Department of Precision Medicine, University of Campania “Luigi Vanvitelli”, Naples, Italy
5 Plastic Surgery Unit, Multidisciplinary Department of Medical-Surgical and Dental Specialties, University of Campania Luigi Vanvitelli,
  Naples, Italy
6 Department of Dermatology and Venereology, University of Zagreb, Zagreb, Croatia



Citation: Caccavale S, Martins Basso A, Vitiello P, et al. Dermatofibrosarcoma protuberans: experience at a third-level referral center.
Dermatol Pract Concept. 2022;12(01):e2022033. DOI: https://doi.org/10.5826/dpc.1201a33
Accepted: August 14, 2021; Published: January 2022
Copyright: ©2022 Caccavale et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License
BY-NC-4.0, which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original authors
and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding author: Stefano Caccavale, MD, Dermatology Unit, Department of Mental and Physical Health and Preventive Medicine,
University of Campania Luigi Vanvitelli, Naples, Italy. Email: stefano85med@libero.it




Dermatofibrosarcoma protuberans (DFSP) is a soft tissue                  staining is characteristically CD34-positive and factor XIIIa
sarcoma,a rare and locally aggressive dermal and subcuta-                negative. Histopathologic features include a dense dermal
neous mesenchymal tumor. DFSP results frequently from the                tumor, the fascicles of which are interspersed with a storiform
translocation t (17;22) with consequent fusion of the genes of           configuration, extending to the adipose tissue. Neoplastic
collagen 1A1 (COLL1A1) and of platelet-derived growth fac-               cells are monomorphous and have hyperchromatic nuclei
tors B (PDGFB) that activate the signaling of tyrosine kinase            [3] (Figure 2).
[1]. DFSP is usually observed between the third and fourth                    The treatment is surgical with a clear histological margin.
decades of life. The distribution between sexes is similar, with         However, the recurrence rate is around 7.3% for excision
a slight predominance in females [2]. DFSP typically presents            with a margin of 2-4 cm. DFSP has low metastatic potential
as a slow-growing, reddish-brown plaque, with protruding                 (2-5%) but a high rate of local recurrence after surgical exci-
firm smooth nodules (Figure 1). The most common sites are                sion. Mohs surgery reduces the recurrence rate (about 1%).
the trunk and proximal extremities. Usually, it is asymptom-             Adjuvant radiotherapy can aid in reducing the local recur-
atic, being less commonly painful. Immunohistochemical                   rence rate [1,3-5]). The use of imatinib reduces the size of




Commentary | Dermatol Pract Concept. 2022;12(01):e2022033                                                                                  1
    Figure 1. (A) Clinical and (B and C) dermoscopic pictures of a DFSP on a man’s deltoid area. On dermoscopy, a brownish or pink
    background can be seen with a pigment network and arborizing vessels.




Figure 2. (A) The neoplastic population is arranged in a storiform pattern, entrapping adipocytes. Cells are spindle-shaped with hyperchromatic
nuclei (hematoxylin and eosin, magnification ×100). (B) The neoplastic cells are positive for CD34 (immunostaining, magnification ×100).


inoperable tumors. Follow-up visits are recommended every                females (52.4%), in accordance with the literature. The mean
3-6 months during the first 3 years [1].                                 age was 44 years for males and 47 years for females. The
     An observational retrospective monocentric study was                diagnosis of DFSP was only part of the clinical differential
conducted. The patient list was extracted from the electronic            diagnosis in 8 patients (Table 2); the most frequent histolog-
database of the Dermatology Unit of the University of Cam-               ical diagnoses in these patients were dermatofibroma and
pania Luigi Vanvitelli, Naples, Italy. Eligible cases affected           basal cell carcinoma (25% each).
by DFSP were identified in the retrospective search in the                   The 21 histologically proven cases of DFSP had involve-
database in the period between January 2006 and November                 ment of the following body areas: scalp, chest and inframa-
2020. Cases lacking adequate clinical information (ie sex, age,          mmary, over surgical scar, back, abdomen, upper limb, groin,
anatomic site, histopathology) were excluded. We identified              lower limb, and foot (Table 1). Among these areas, the chest
29 cases with a clinical suspicion for DFSP (Table 1). Among             was the most frequently affected by the tumor (33.3%) fol-
them, 21 were confirmed by histological examination (10                  lowed by the back and abdomen (14.3%, each).
men aged 23-68 years at the time of diagnosis, and 11 women                  Six patients did not return after the first visit or after
25-79 years old). We found a slightly higher prevalence in               surgery. Some of them had already had a biopsy in another



2                                                                              Commentary | Dermatol Pract Concept. 2022;12(01):e2022033
                     Table 1. Body Areas Affected by DFSP in Histologically Proven Cases
                     Number of                                                                             Number of Cases for Each
                       Cases           Female           Age               Male             Age                   Body Area
 Histologically      21             11              25-79            10                 23-68          •   7 chest
 proven cases of                                                                                       •   3 abdomen
 DFSP                                                                                                  •   3 back
                                                                                                       •   2 lower limb
                                                                                                       •   2 upper limb
                                                                                                       •   1 over surgical scar
                                                                                                       •   1 groin
                                                                                                       •   1 foot
                                                                                                       •   1 scalp

DFSP = dermatofibrosarcoma protuberans.



Table 2. Patients With Clinical Suspicion of DFSP and Their “True” Diagnoses After Histopathologic
                                             Examination
                                                                                    Number of Patients for Each Histopathologic
                                                    Number of Cases                                 Diagnosis
 Clinical suspicion of DFSP                   29 (21 confirmed with HPE)            21
 Clinical suspicion of DFSP not               8                                     •   2 dermatofibroma
 confirmed with histopathology                                                      •   2 basal cell carcinoma
                                                                                    •   1 invasive melanoma
                                                                                    •   1 squamous cell carcinoma
                                                                                    •   1 hypertrophic scar
                                                                                    •   1 trichilemmal cyst

DFSP = dermatofibrosarcoma protuberans; HPE = histopathologic examination.



hospital and came to us asking for a second opinion. Among                2. Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibro-
the 13 cases that returned only once for the follow-up visit,                sarcoma protuberans. Cancer. 2004;101(11):2503-2508. DOI:
                                                                             10.1002/cncr.20678. PMID: 15503305.
1 patient was also affected by a melanoma and another one
                                                                          3. Tan AW, Tan SH. Dermatofibrosarcoma protuberans: a clinico-
returned for management of urticaria. Only 1 patient under-
                                                                             pathological analysis of 10 cases in Asians. Australas J Dermatol.
went follow-up controls for more than 5 years.                               2004;45(1):29-33. DOI: 10.1111/j.1440-0960.2004.00048.x.
   The number of DFSP diagnosed in our Dermatology                           PMID: 14961905.
Unit between January 2006 and November 2020 is certainly                  4. Ratner D et col. Mohs micrographic surgery for the treatment of
not high, especially when compared to the huge number of                     dermatofibrosarcoma protuberans. Results of a multiinstitutional
                                                                             series with an analysis of the extent of microscopic spread. J Am
patients that we see every day in our third-level referral center.
                                                                             Acad Dermatol. 1997;37(4):600-613. DOI: 10.1016/s0190-
However, as DFSP is a rare tumor, our data are not irrelevant.
                                                                             9622(97)70179-8. PMID: 9344201.
We have noticed difficulty in monitoring patients because                 5. Criscione VD, Weinstock MA. Descriptive epidemiology of
they did not return after evaluation or after surgical removal.              dermatofibrosarcoma protuberans in the United States, 1973 to
                                                                             2002. J Am Acad Dermatol. 2007;56(6):968-973. DOI: 10.1016/j.
                                                                             jaad.2006.09.006. PMID: 17141362.
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    10.1245/s10434-007-9480-y. PMID: 17647063.




Commentary | Dermatol Pract Concept. 2022;12(01):e2022033                                                                                    3
