Indian Journal of Dermatology
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E-IJD-CASE REPORT
Year : 2014  |  Volume : 59  |  Issue : 6  |  Page : 635
Dermatofibrosarcoma protuberans at an uncommon site


Department of Dermatology, Venereology and Leprosy, Bharati Vidyapeeth University Medical College and Hospital, Pune, India

Date of Web Publication30-Oct-2014

Correspondence Address:
Vidyadhar R Sardesai
102 Alliance Nakshatra, 48 Tulshibagwale Colony, Sahakar Nagar No. 2, Pune - 411 009
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.143602

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   Abstract 

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive, cutaneous, malignant tumor characterized by high propensity for local relapse and low metastatic potential. It is seen mainly in the age group of 6-65 years over the trunk and extremities. Immunohistochemically it diagnosed by marker human progenitor cell Ag CD 34. Herein, we present a 40-year-old male with DFSP over the pubic area and extending up to the proximal penile shaft. The biopsy revealed intervening bundles of spindle-shaped cells in the dermis and the diagnostic marker human progenitor cell Ag CD 34 was positive. The patient underwent wide local surgical resection.


Keywords: CD34, dermatofibrosarcoma protuberans, soft tissue sarcoma


How to cite this article:
Sardesai VR, Patil RM, Agarwal TD. Dermatofibrosarcoma protuberans at an uncommon site. Indian J Dermatol 2014;59:635

How to cite this URL:
Sardesai VR, Patil RM, Agarwal TD. Dermatofibrosarcoma protuberans at an uncommon site. Indian J Dermatol [serial online] 2014 [cited 2020 Jan 26];59:635. Available from: http://www.e-ijd.org/text.asp?2014/59/6/635/143602

What was known?
DFSP is locally aggressive fi brous tumor affecting the trunk and extremities.



   Introduction Top


Dermatofibrosarcoma protuberans (DFSP) is a rare slow growing skin tumor with tendency to local recurrence. [1] It is a locally aggressive fibrous tumor but rarely shows metastasis to local lymph nodes or distant sites. Hallmark of the disease is characteristic arrangement of spindle-shaped tumor cells in dermis. [2] Positive staining for CD34 on immunnohistochemistry is also pathgnomic. [3] Wide and deep local excision is the treatment of choice. DFSP is rare and represents 1-6% of all soft tissue sarcomas. The commonly affected areas are the trunk, extremities, scalp, and neck. Only one case has been reported in the suprapubic area. This report describes a case of suprapubic DFSP involving the proximal penile shaft.


   Case Report Top


A 40-year-old male, farmer by occupation, presented with complaints of foul smelling lesions over the pubic region since 7 months. He was symptomless for the first 6 months after history of similar lesions 1 year back, which were excised. There was no history of sexually transmitted infection and diabetes mellitus. General and systemic examination was normal.

Examination revealed three erythematous fleshy nodules measuring about 3-4 cm in size with foul smelling discharge over the pubic area extending to the proximal shaft of the penis [Figure 1]. The surface of the swelling showed multiple protruberations with nodules, which were firm, non tender, freely mobile over the underlying structure. The distal penile shaft and scrotum were spared. No regional lymphadenopathy was seen.

All routine investigations were within normal limits. Test for human immunodeficiency virus (HIV), syphilis, hepatitis B were negative. Biopsy of the lesion revealed flattening of epidermis in one area and elongated rete pegs in the other parts. In the dermis, a tumor composed of intervening bundles of spindle-shaped cells was seen [Figure 2]. The immunohistochemical marker human progenitor cell Ag CD 34 was positive.
Figure 1: Three erythematous fleshy nodules measuring about 3-4 cm in size over the pubic area extending to the proximal shaft of the penis

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Figure 2: Histopathology shows characteristic spindle shape tumor cells in the dermis (H and E × 10)

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A diagnosis of DFSP was made and treated by wide local excision with 2 cm of free margin. [Figure 3].
Figure 3: Wide local excision with 2 cm of free margin

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   Discussion Top


DFSP is a locally aggressive, cutaneous, malignant tumor characterized by high propensity for local relapse and low metastatic potential. DFSP have been reported in the literature as early as 1890, Darier and Ferrand first described it in 1924 as a distinct cutaneous disease entity called progressive and recurring dermatofibrosarcoma. [4] Hoffman officially coined the term DFSP in 1925. [5] DFSP represents 1-6% of all soft tissue sarcomas. [6]

Age of onset of the disease ranges from 6 to 65 years. [1] It usually involves the trunk, proximal extremities and the scalp. Head, necks and genitals are rarely involved, [7] whereas in our case the proximal penile shaft was involved. The tumor tends to appear first as a single firm, fibrous nodule in skin, with a pink or violaceous hue, which is freely mobile on deeper structures. It enlarges by expansile growth, while the periphery of tumor infiltrates the adjacent skin and subcutaneous tissue. Differential diagnosis in the initial stages should include lipomas, epidermal cysts, keloid, and nodular fasciitis. In late stages, when it becomes protuberant, it should be differentiated from pyogenic granuloma and other soft tissue sarcomas.

Immunohistochemically, most DFSP stain positively for CD34, [3] which was also positive in our case. Histopathologically it, is characterized by the arrangement of spindle-shaped tumor cells in a "cartwheel" pattern, [2] cytologically monomorphous bland spindle cells, with a characteristic finger-like, honeycomb pattern of infiltration into the subcutaneous fat.

The treatment of choice is wide surgical excision ensuring adequate margins of 3-5 cm, followed by immediate reconstruction. [8] Radiotherapy and chemotherapy have been tried with limited success . The significant prognostic factor for relapse is considered to be the extent of the initial resection as close margins that is less than 2 cm shows a statistically significant positive correlation with recurrence. [9] Mohs micrographic surgery is the precise margin controlled approach for excision of DFSP. [10]

 
   References Top

1.Brenner W, Schaefler K, Chhabra H, Postel A:Dermatofibrosarcoma protuberans metastatic to a regional lymph node. Report of a case and review. Cancer 1975;36:1897-1902.  Back to cited text no. 1
    
2.Taylor HB, Helwig EB: Dermatofibrosarcoma protuberans. A study of 115 cases. Cancer 1962;15:717-725.  Back to cited text no. 2
    
3.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;22:576-587.  Back to cited text no. 3
    
4.Criscione VD, Weinstock MA. Descriptive epidemiology of Dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermato 2007;56:968-73.  Back to cited text no. 4
    
5.Hoffman E: Uber das knollentreibende fibrosarkom der haut. Dermatol Zischr 1925;43:1-28.  Back to cited text no. 5
    
6.Kransdorf MJ: Malignant soft-tissue tumors in a large referral population: Distribution of diagnoses by age, sex, and location. AJR Am J Roentgenol 1995;164:129-134.  Back to cited text no. 6
    
7.Leake JF, Buscema J, Cho KR, et al. Dermatofibrosarcoma protuberans of the vulva. Gynecol Oncol. 1991;41:245-9.  Back to cited text no. 7
    
8.Lobay GW, Bryce Weir, Robert Carter: DFP of the scalp treated by radical excision, emmediate cranioplasty, and free groin flap: Case report. J Neurosurg 1981;55:640-642.  Back to cited text no. 8
    
9.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. 9
    
10.Dermatol Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for Dermatofibrosarcoma protuberans: University of Miami and NYU experience. Surg 2002;28:1060-4.  Back to cited text no. 10
    

What is new?
Involvement of proximal penile shaft with suprapubic region by DFSP.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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    Abstract
   Introduction
   Case Report
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    References
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