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  Table of Contents  
LETTER TO THE EDITOR
Year : 2017  |  Volume : 8  |  Issue : 6  |  Page : 495-497  

Dermatofibrosarcoma protuberans


1 Department of Dermatology, MNR Medical College and Hospital, Sangareddy, Telangana, India
2 Department of Dermatology, Gadag Institute of Medical Sciences, Gadag, Karnataka, India

Date of Web Publication14-Nov-2017

Correspondence Address:
Shashikant Malkud
Department of Dermatology, MNR Medical College and Hospital, Sangareddy, Telangana - 502 294
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_424_16

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How to cite this article:
Malkud S, Dyavannanavar V. Dermatofibrosarcoma protuberans. Indian Dermatol Online J 2017;8:495-7

How to cite this URL:
Malkud S, Dyavannanavar V. Dermatofibrosarcoma protuberans. Indian Dermatol Online J [serial online] 2017 [cited 2019 Aug 21];8:495-7. Available from: http://www.idoj.in/text.asp?2017/8/6/495/218346



Sir,

A 42-year-old woman presented with history of multiple asymptomatic progressive nodular lesions over abdomen since 8 years. Patient had small nodular lesion at the same site, which was excised 9 years back. On cutaneous examination, multiple erythematous nodules of variable sizes were coalescing to form a large plaque over the abdomen [Figure 1]. A large solitary erythematous protuberant nodule measuring 10 × 8 cm was present over the plaque. Scaling and crusting were noticed over larger lesion. On palpation it was firm, non-tender, and not freely mobile over the underlying structures. There was no regional lymphadenopathy. On investigation, her complete blood count, liver function tests, and renal function tests were within normal limit. Serological tests for human immunodeficiency virus (HIV) and hepatitis B were negative. An incisional biopsy was performed under local anesthesia and tissue was sent for histopathology. Hematoxylin and eosin (H and E) stain showed spindle shaped tumor cells arranged in storiform pattern in the dermis [Figure 2]. Immunohistochemical (IHC) study showed positive for human progenitor cell Ag CD34 [Figure 3] and vimentin and negative for S100 protein, features suggestive of dermatofibrosarcoma protuberans (DFSP).
Figure 1: Multinodular mass with protuberant lesion over abdomen

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Figure 2: Histopathology showing spindle shape tumor cells in dermis (H and E, ×10)

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Figure 3: Immunohistochemistry showing positivity to CD34 (IHC, ×40)

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DFSP is a locally aggressive, low grade, and relatively uncommon cutaneous neoplasm which has a high propensity for local relapse with low metastatic potential.[1],[2] It accounts for <0.1% of all neoplasms, with an annual reported incidence of 0.8–4.5 cases per million.[3],[4] It is most commonly seen in third or fourth decade with slight male preponderance.[5] Trunk is the most common site affected followed by proximal extremities and scalp. Head and neck, and genitals are rarely involved.[6] Clinically, it presents as pink or violet red plaque which may be surrounded by telangiectatic skin.[7] These lesions are fixed to skin, but move freely over underlying structures. They do not exhibit nodular growth pattern until late in their course. It becomes fixed to underlying structures in advanced and/or recurrent cases.[8] Clinically, it may be confused with other conditions such as sclerosing basal cell carcinoma, morphea, scar, and anetoderma.[8],[9] On histopathology, it shows spindle-shaped neoplastic cells arranged in cartwheel or storiform pattern in the dermis.[7] Immunohistochemical stains are employed to differentiate DFSP from dermatofibroma, as sometimes it is difficult to differentiate the two on routine H and E stain. Studies have shown that CD-34 and vimentin are usually expressed in many cases of DFSP. Coagulation factor XIIIa is generally not expressed in DFSP, but is strongly expressed in dermatofibroma.[10] In our case it showed positive for both CD34 and vimentin. Surgical excision is the treatment of choice. Due to the high recurrence rate, radical excision is the preferred option. It involves complete surgical excision of the tumor with wide margins (>3 cm).[7] Mohs micrographic surgery is the most precise margin controlled approach for excision of DFSP.[11]

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Krishnamurthy A, Majhi U. Fibrosarcomatous dermatofibrosarcoma protuberans: An unusual tumor in the facial skin. Indian J Dermatol 2014;59:105.  Back to cited text no. 1
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2.
Cotoi OS, Muresan AV, Tilinca MC, Chiotoroiu AL, Badea MA, Suciu M, et al. Giant dermatofibrosarcoma protuberans-rare form of mesenchymal tissue neoplasm: Case presentation. Rom J Morphol Embryol 2014;55:1491-5.  Back to cited text no. 2
    
3.
Criscione VD, Weinstock MA. Descriptive epidemiology of Dermatofibrosarcoma protuberans in the United States, 1973 to2002. J Am Acad Dermatol 2007;56:968-73.  Back to cited text no. 3
    
4.
Stojadinovic A, Karpoff HM, Shah JP, Singh B, Spiro RH, Shaha AR, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol 2000;7:696-704.  Back to cited text no. 4
    
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Bhambri S, Desai A, Del Rosso JQ, Mobini N. Dermatofibrosarcoma Protuberans A Case Report and Review of the Literature. J Clin Aesthet Dermatol 2008;1:34-6.  Back to cited text no. 5
    
6.
Leake JF, Buscema J, Cho KR, Currie JL. Dermatofibrosarcoma protuberans of the vulva. Gynecol Oncol 1991;41:245-9.  Back to cited text no. 6
    
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Sardesai VR, Patil RM, Agarwal TD. Dermatofibrosarcoma protuberans at an uncommon site. Indian J Dermatol 2014;59:635.  Back to cited text no. 7
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Laskin WB. Dermatofibrosarcoma protuberans. CA Cancer J Clin 1992;42:116-25.  Back to cited text no. 8
    
9.
Arifin MZ, Yudoyono F, Dahlan RH, Hernowo BS, Sutiono AB, Faried A. A rare giant scalp dermatofibrosarcoma protuberans. Surg Neurol Int 2014;5:45.  Back to cited text no. 9
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10.
Liansheng L, Xialiang L, Yaodong Z, Yajun X, Meiqing L. Report of two cases of recurrent scalp dermatofibrosarcoma protuberans and literature review. Indian J Dermatol 2014;59:602-5  Back to cited text no. 10
    
11.
Nouri K, Lodha R, Jimenez G, Robins P. Mohs micrographic surgery for Dermatofibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg 2002;28:1060-4.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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