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Recurrent dermatofibrosarcoma protuberans: A report of two cases involving the trunk and abdominal wall

1 Department of Surgery, Maulana Azad Medical College, Lok Nayak Hospital, New Delhi, India
2 Department of General Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Date of Submission20-Jan-2021
Date of Decision03-Apr-2021
Date of Acceptance18-Apr-2021
Date of Web Publication15-Oct-2021

Correspondence Address:
Tusharindra Lal,
Sri Ramachandra Institute of Higher Education and Research, Chennai - 600 116, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.jcrt_125_21

 > Abstract 

In this paper, we report two cases of dermatofibrosarcoma protuberans (DFSP) who were treated with distinct surgical approaches. In the first case, a 50-year-old woman presented with a mass on her right shoulder and underwent local excision with subsequent reconstruction using a deltopectoral flap. The second case was of a young female who presented with a giant protuberant DFSP on the anterior abdominal wall who was treated with wide local excision along with inlay mesh repair of the defect. Early excision and adjuvant radiotherapy facilitates a low recurrence rate while also improving the prognosis of the patients.

Keywords: Deltopectoral flap, dermatofibrosarcoma protuberans, recurrence, wide local excision

How to cite this URL:
Vindal A, Lal T, Gupta Y, Kale AR, Lal P. Recurrent dermatofibrosarcoma protuberans: A report of two cases involving the trunk and abdominal wall. J Can Res Ther [Epub ahead of print] [cited 2022 Jun 25]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=328257

 > Introduction Top

Dermatofibrosarcoma protuberans (DFSP) is a locally invasive, mesenchymal tumor with a high recurrence rate and may involve multiple layers of the skin including the dermis, subcutaneous fat and may even incorporate the underlying muscles and fascia.[1],[2],[3] With this paper, the authors want to emphasize the importance of recurrence of DFSP even with clear margins on histopathology and report two cases of DFSP involving the trunk and anterior abdominal wall presenting in an advanced local stage which were managed with different surgical techniques.

 > Case Reports Top

Case report 1

A 50-year-old female presented with a rapidly progressive swelling on her right shoulder for the past 4 months. She had previously undergone a local excision a few months back. On physical examination, a large lobulated ulceroproliferative growth measuring 10 cm × 7 cm was reported on the right supraclavicular region [Figure 1]a. With a provisional diagnosis of soft-tissue sarcoma, an incisional biopsy was performed. The biopsy revealed homogenous, spindle-shaped cells in a storiform and cartwheel pattern. A focus of giant cells was identified and was occasionally seen to entrap fat. On immunohistochemistry (IHC), tumor cells were seen to express CD34 and vimentin which was typical of DFSP. A preoperative contrast-enhanced computed tomography (CECT) of the head and the neck confirmed a lesion in the subcutaneous plane with no evidence of distant spread.
Figure 1: (a) Ulceroproliferative lesion in the right pectoral region. (b) Postoperative image showing reconstruction using a deltopectoral fasciocutaneous flap

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A wide local excision was performed and the resultant triangular defect was covered by a cranially and medially rotated fasciocutaneous deltopectoral (DP) flap [Figure 1]b. The pliability of the skin resulted in tensionless suturing. Skin sutures were removed after 2 weeks, and the patient was subjected for postoperative radiotherapy. The patient is currently well after 4 years of follow-up without any evidence of local or distant recurrence.

Case report 2

A 20-year-old, thin-built female patient presented with a dull-aching right upper abdominal swelling which progressively increased in size from 2 cm × 2 cm to 20 cm × 15 cm in the last 3 months. The patient also had a history of anorexia and weight loss. On examination, a large protuberant swelling with an overlying ulcer of 2 cm × 4 cm was seen occupying the right hypochondrium, lumbar and extending into the epigastric region [Figure 2]a. The swelling was hard in consistency and was mobile over the abdominal wall muscles. Magnetic resonance imaging [Figure 2]b showed a subcutaneous lesion with no evidence of metastases on CECT chest and abdomen. Core biopsy was consistent with features of DFSP with positivity for CD34 and vimentin on IHC.
Figure 2: (a) A large protuberant swelling in the right hypochondrium and lumbar region. (b) Magnetic resonance imaging showing involvement of all layers of the muscles of abdominal wall with intact peritoneum. (c) Resected specimen with a 5-cm margin of clearance on all sides. (d) Recurrent tumor with scar of previous surgery

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At surgery, the tumor was found to invade only the external oblique aponeurosis (EOA) and muscle while sparing the rest of the abdominal muscles which were both excised, keeping a 2-cm margin. The defect of the EOA was bridged using a 15 cm × 15 cm polypropylene mesh in inlay fashion under the cut edges of the EOA/muscle and deeper muscles. Skin flaps were approximated primarily with adequate mobilization. Histopathological examination (HPE) of the specimen [Figure 2]c confirmed DFSP with pleomorphic spindle cells having moderate cytoplasm and an elongated hyperchromatic nucleus with clear radial and deep resected margins up to 1 cm. After an uneventful recovery, she was planned for postoperative radiotherapy but was lost to follow-up until 6 months later when she presented with a recurrent swelling at the scar line [Figure 2]d. The swelling was re-excised along with the mesh, and the defect was closed with primary repair and clear excision margins on HPE. The patient is currently undergoing radiotherapy and chemotherapy and is asymptomatic 1 year after the second surgery.

 > Discussion Top

DFSP is an intermediate-grade, malignant tumor which originates from dermal stem cells or undifferentiated mesenchymal cells. It accounts for only 2–6% of all soft-tissue sarcomas.[1] The tumor usually has a low metastatic rate but has a high preponderance for local recurrence. While some cases suggest equal proportions of sex distribution, DFSP is commonly seen in females with a median age of 35 years.[4] In majority of the cases, the tumor tends to involve the trunk (42%–72%), proximal extremities (16%–30%), and the head-and-neck regions (10%–16%).[1],[5],[6] Some of the risk factors associated with this tumor include previous surgical interventions, skin trauma, vaccination, tattooing, radiation, burns, pregnancy, and hormonal treatment with estrogen.[3],[5] The conversion of this tumor to a high-grade fibrosarcomatous component is rarely seen with over 90% of cases presenting with low-to-intermediate-grade malignancy.[6],[7]

The pathogenesis of the tumor results from a chromosomal translocation t(17;22) (q22;q13) resulting in the fusion of collagen type Iα1 gene (COL1A1) and platelet-derived growth factor-beta polypeptide gene (PDGFB) leading to the formation of COL1A1-PDGFB fusion transcripts. This causes the upregulation of PDGFB and continuous activation of platelet-derived growth factor receptor-beta leading to proliferation of cells and tumor formation.[8]

On gross appearance, DFSP shows a reddish-brown to normal skin color, thickened plaque which gradually becomes nodular and turns into a protuberant tumor. On HPE, classical storiform and cartwheel arrangement of spindle cells is seen with little or no mitosis along with CD34 positivity on IHC.[5]

The preferred treatment modality for DFSP is the Mohs micrographic surgery which helps in thorough histological clearance with a low recurrence rate of 1%. Majority of the surgeons recommend an excision with a margin of 2–4 cm around the surrounding skin, including underlying fascia without elective lymph node dissection.[7],[9]

Skin defects after wide local excision may be corrected with the help of local or distant flaps. In the first case, a DP flap reconstruction was performed based on the perforators of second and third internal mammary arteries.[10] Abdominal wall skin defects can be covered using local skin mobilization or with transverse rectus abdominis myocutaneous flap. The former restricts wide excision margin desired for such cases (2–4 cm), and therefore, flaps are desirable wherever possible. Prosthetic abdominal wall defect repair helps to restore abdominal wall strength where part of the EOA or sheath is excised to prevent postoperative incisional hernia.[3] The possible reasons for recurrence in the second case could be due to nonusage of flap, thus compromising excision margin and missing out on radiotherapy due to losing follow-up.

DFSP is a radioresponsive tumor where combined conservative resection and postoperative radiation should be considered when an adequate wide excision alone may result in a major cosmetic or functional deficit.[11] The tumor has a good overall prognosis of 99.1% with a 10-year survival rate.[12] A close follow-up of at least 3 years is advised due to higher chances of recurrence in this time period.

 > Conclusion Top

DFSP is a locally invasive tumor with a high recurrence rate. In spite of the size of the tumor, it can be effectively treated with appropriate surgical techniques. Early surgical excision with wide local margins of at least 2 cm and prompt radiotherapy facilitated with a dedicated follow-up period reduces the recurrence rate and improves the morbidity as well as mortality.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Kar SK, Recurrent fibrosarcoma protuberans of anterior chest and abdominal wall. A rare case report. Arch Med. 2015,8:1.  Back to cited text no. 1
Chang CK, Jacobs IA, Salti GI. Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol 2004;30:341-5.  Back to cited text no. 2
Tropea S, Mocellin S, Stramare R, Bonavina MG, Rossi CR, Rastrelli M. Desmoid Fibromatosis of the Abdominal Wall: Surgical Resection and Reconstruction with Biological Matrix Egis®. Case Rep Oncol 2017;10:205-11.  Back to cited text no. 3
Kreicher KL, Kurlander DE, Gittleman HR, Barnholtz-Sloan JS, Bordeaux JS. Incidence and survival of primary dermatofibrosarcoma protuberans in the United States. Dermatol Surg 2016;42 Suppl 1:S24-31.  Back to cited text no. 4
Wiesmueller F, Agaimy A, Perrakis A, Arkudas A, Horch RE, Grützmann R, et al. Dermatofibrosarcoma protuberans: Surgical management of a challenging mesenchymal tumor. World J Surg Oncol 2019;17:90.  Back to cited text no. 5
Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD. Fibrosarcomatous (”high-grade”) dermatofibrosarcoma protuberans: Clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol 1998;22:576-87.  Back to cited text no. 6
Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM, et al. Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution. Cancer 2000;88:2711-20.  Back to cited text no. 7
Takahira T, Oda Y, Tamiya S, Higaki K, Yamamoto H, Kobayashi C, et al. Detection of COL1A1-PDGFB fusion transcripts and PDGFB/PDGFRB mRNA expression in dermatofibrosarcoma protuberans. Mod Pathol 2007;20:668-75.  Back to cited text no. 8
Fiore M, Miceli R, Mussi C, Lo Vullo S, Mariani L, Lozza L, et al. Dermatofibrosarcoma protuberans treated at a single institution: A surgical disease with a high cure rate. J Clin Oncol 2005;23:7669-75.  Back to cited text no. 9
Mortensen M, Genden EM. Role of the island deltopectoral flap in contemporary head and neck reconstruction. Ann Otol Rhinol Laryngol 2006;115:361-4.  Back to cited text no. 10
Ballo MT, Zagars GK, Pisters P, Pollack A. The role of radiation therapy in the management of dermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys 1998;40:823-7.  Back to cited text no. 11
Wollina U, Langner D, Schönlebe J, França K, Lotti T, Tchernev G. Dermatofibrosarcoma protuberans: Retrospective single center analysis over 16 years. Open Access Maced J Med Sci 2018;6:35-7.  Back to cited text no. 12


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