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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1038

Giant dermatofibrosarcoma protuberans: A rare presentation over face

1 Department of General Surgery, VMMC and Safdarjung Hospital, New Delhi, India
2 Department of Oral Medicine and Radiology, Institute of Dental Studies and Technologies, Kadrabad, Modinagar, Uttar Pradesh, India

Date of Web Publication15-Feb-2016

Correspondence Address:
Lokendra Kumar
Department of General Surgery, VMMC and Safdarjung Hospital, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.139386

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 > Abstract 

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive, soft tissue tumor. Its occurrence over face is very rare. We are presenting a case of giant DFSP over left cheek, which produces oncological, functional and esthetic challenges and deals by multidisciplinary team. We also reviewed the relevant literature in short.

Keywords: Dermatofibrosarcoma protuberans, pectoralis major myocutaneous flap, mohs micrographic surgery, wide local excision

How to cite this article:
Kumar L, Bhandari V, Singh S, Garg P, Kumar A. Giant dermatofibrosarcoma protuberans: A rare presentation over face. J Can Res Ther 2015;11:1038

How to cite this URL:
Kumar L, Bhandari V, Singh S, Garg P, Kumar A. Giant dermatofibrosarcoma protuberans: A rare presentation over face. J Can Res Ther [serial online] 2015 [cited 2020 Jul 2];11:1038. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1038/139386

 > Introduction Top

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor. It is characterized by high rates of local recurrence and low risk of metastasis. [1,2] DFSP occurs most commonly on the trunk and proximal extremities. [3,4] The occurrence of this tumor on the face is extremely rare but local recurrence is highest over the head and face.

 > Case Report Top

This was a case report of 52 year old gentlemen who presented with the complaint of large mass lesion over the left side of face since 2 years. On examination he was anemic, averagely nourished and rest parameters were in normal range. The lesion was around 20 X 18 cm large, lobulated, hard in consistency, protruding and extending supero-inferiorly lower eyelid to upper border of mandible, antero-posteriorly left side of nose to tragus of ear and also involving left upper and lower lip with angle of mouth. Multiple dilated cutaneous veins; ulceration and scabbing were presented over prominent part of the lesion [Figure 1]. Multiple lymph nodes were palpated in level I and II of left side neck. Investigation revealed hemoglobin 7.3g/dl, all other laboratory studies including liver function and chest X-ray was reported normal. Computed tomography (CT) of head neck and face revealed 15 X 10 X 13.7 cm size, solid, lobulated, exophytic soft-tissue mass lesion, arising from subcutaneous plain in left maxillofacial region, homogenously enhancing with a lack of enhancement in central region suggestive of necrosis, fat plain with buccinator and masseter muscle was lost [Figure 2] and multiple lymph nodes at level I and II, largest was 1.5 X 1.2 cm. Incisional biopsy revealed fibrohistiocytic lesion. Pre-operatively, he was adequately optimized. He was operated under general anesthesia in supine position by a multidisciplinary team. Wide local excision of lesion with selective lymph node dissection (Level I-III) with reconstruction of primary defect using supratrochlear based flap for inner lining and pectoralis major myocutaneous flap for outer covering with covering of secondary defect by superficial skin graft taken from left thigh [Figure 3]. He was treated in intensive care unit and post- operative period was uneventful [Figure 4]. Histopathological examination of the completely excised tumor revealed highly cellular tumor consisting of spindle cells arranged in storiform pattern and in some places herring bone pattern can also be recognized. Cells were showing severe degree of pleomorphism, cytoplasm of many cells were foamy and nucleus were varies vesicular to hyper chromic, skin of peripheral region and deeper resected margin was free of tumor cells and all of these features were suggestive of DFSP. A total of 11 lymph nodes were isolated from level I-III and all were showing reactive lymphadenitis. After discussion with medical oncologist and radiotherapist, he was transfer to medical oncology department for adjuvant chemotherapy.
Figure 1: Giant dermatofibrosarcoma protuberans over left side of the face with dilated veins and scabbing over prominent part of lesion

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Figure 2: Computed tomography images showing soft-tissue tumor mass along with the extensions

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Figure 3: Resected specimen and reconstruction of the primary defect by pectoralis major myocutaneous flap

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Figure 4: Post-operative image after 1 month

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 > Discussion Top

DFSP is a rare, fibrohistiocytic tumor. The estimated incidence of DFSP is 4.5 cases/million persons/year in the USA.[5] DFSP first described by Sherwel[6] and Taylor[7], independently. Hoffman[8] coined the term DFSP. DFSP most commonly occurs between 20 and 50 years of age, although its can appear at any age. The most frequently affected region is the trunk, nearly 40-50% followed by the upper and lower extremities 30-40%. [3,4] Head and neck cases account for approximately 10-15% of cases.[9] The occurrence of this tumor on the face is extremely rare. DFSP initially appears as an asymptomatic, indurated plaque that may have a violaceous, red-blue, or brown appearance, with a hard consistency and fixed to the skin but not to the underlying structure. However, recurrent or long-standing tumors may invade fascia, striated muscle, periosteum, and bone.[10] Over a period, which can vary from a few months to decades, the DFSP grows with the development of multiple nodules within the plaque, from which its name protuberans is derived. DFSP appears as a poorly circumscribed tumor that infiltrate the whole dermis destroying the preexisting structures and spreading into the cellular subcutaneous tissue. The tumor is composed predominantly of a dense, uniform array of cells with spindle-shaped nuclei embedded in varying amounts of collagen. This fibroblast-like proliferation is generally arranged into irregular, interwoven fascicles, resulting in a storiform pattern. In addition to physical examination, Magnetic resonance imaging or CT may be useful in evaluating extent of tumor spread. The goal remains complete excision of tumor cells with maximum preservation of normal tissue to maintain function and an acceptable aesthetic appearance. The need for clear margins on the face creating potentially large tissue defects poses many reconstructive challenges. Mohs Micrographic Surgery or staged wide excision “Slow Mohs” (with formal histopathological sectioning and delayed reconstruction for complete circumferential peripheral and deep margin assessment) is becoming the standard surgical treatment for DFSP.[11] The current recommendations of surgical margins is at least 2 cm, preferably 3 cm.[12] Local recurrence rate is between 32% and 76%.[13] However, metastatic disease is rare; it has been reported in only 4% of cases. Targeted molecular therapy aims at arresting tumor growth by blocking platelet-derived growth factor (PDGF) activity. Imatinib mesylate is a potent and selective tyrosine kinase inhibitor, with efficacy against the PDGF receptors.[11] It is used for cases that are unresectable, recurrent or metastatic. Adjuvant radiation therapy has proven benefit in incompletely excised tumors.[14] However, its use is controversial. Chemotherapy has no proven benefit in locally aggressive DFSP and its efficacy in metastatic disease is undefined. We are reporting a case of DFSP with unusually giant occurrence over face. He was treated by wide local excision with primary reconstruction.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

 > References Top

Gloster HM-Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol 1996;35:355-74.  Back to cited text no. 1
Sanmartin O, Llombart B, Lopez-Guerrero JA, Serra C, Requena C, Guillen C. Dermatofibrosarcoma protuberans. Actus Dermosifilioger 2007;98:77-78.  Back to cited text no. 2
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. 3
McPeak CJ, Cruz T, Nicastri AD. Dermatofibrosarcoma protuberans: An analysis of 86 cases – Five with metastasis. Ann Surg 1967;166:803-16.  Back to cited text no. 4
Rouhani P, Fletcher CD, Devesa SS, Toro JR. Cutaneous soft tissue sarcoma incidence patterns in the U.S.: An analysis of 12,114 cases. Cancer 2008;113:616-27.  Back to cited text no. 5
Sherwell S, Morphea. Arch Dermatol 1890;8:72-3.  Back to cited text no. 6
Taylor RW. Sarcomatous tumors resembling in some respects keloids. Arch Dermatol 1890;8:384-7.  Back to cited text no. 7
Hoffman E. Dermatofibrosarcoma Protuberans. Dermatol Z 1925;43:1-28.  Back to cited text no. 8
Stojadinovic A, Karpoff HM, Antonescu CR, Shah JP, Singh B, Spiro RH, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol 2000;7:696-704.  Back to cited text no. 9
Barnes L, Coleman JA Jr, Johnson JT. Dermatofibrosarcoma protuberans of the head and neck. Arch Otolaryngol 1984;110:398-404.  Back to cited text no. 10
Llombart B, Serra-Guillén C, Monteagudo C, López Guerrero JA, Sanmartín O. Dermatofibrosarcoma protuberans: A comprehensive review and update on diagnosis and management. Semin Diagn Pathol 2013;30:13-28.  Back to cited text no. 11
Laskin WB. Dermatofibrosarcoma protuberans. CA Cancer J Clin 1992;42:116-25.  Back to cited text no. 12
Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E. Dermatofibrosarcoma protuberans: Treatment and prognosis. Eur J Surg Oncol 1992;18:241-8.  Back to cited text no. 13
Suit H, Spiro I, Mankin HJ, Efird J, Rosenberg AE. Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol 1996;14:2365-9.  Back to cited text no. 14


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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