Dermatofibrosarcoma protuberans with pit-like lesions: A case report and literature review

Jia,Jinjing; Zheng,Yan; Dong,Xinyu; Wang,Xin; Yang,Jiao; Ying,Zhaoxia; Wang,Yongxian; Niu,Xinwu; Xiao,Shengxiang

doi:10.3892/ol.2015.3741

Dermatofibrosarcoma protuberans with pit-like lesions: A case report and literature review

Authors:
- Jinjing Jia
- Yan Zheng
- Xinyu Dong
- Xin Wang
- Jiao Yang
- Zhaoxia Ying
- Yongxian Wang
- Xinwu Niu
- Shengxiang Xiao
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Affiliations: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710004, P.R. China
Published online on: September 24, 2015 https://doi.org/10.3892/ol.2015.3741
Pages: 3765-3768

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Abstract

The present study describes a case of pit-like dermatofibrosarcoma protuberans (DFSP) with the clinical manifestations, histopathological features and criteria for diagnosis. The study also reviews the relevant literature in order to raise awareness among dermatologists with regard to the specific behavior of DFSP. A 27‑year‑old female presented with subcutaneous nodules on the left side of the neck that had been apparent for 5 years and which had gradually begun caving in during the last year. Histopathological examination revealed that the tumor was composed of a large number of spindle cells arranged in a whirlpool‑like pattern. Immunohistochemical studies revealed positive staining for cluster of differentiation 34, vimentin and lysozyme, which could be used as diagnostic markers of DFSP. The patient was finally diagnosed with DFSP by pathological and immunohistochemical analyses. The DFSP was treated with an extended resection followed by adjacent skin repair. The patient responded well and no relapse occurred during the 8‑month clinical follow‑up. Thus, the current study describes a unique pit‑like clinical manifestation of DFSP with typical immunohistochemical and pathological features. In addition, histopathological examination revealed a downward depression in the epidermis. Therefore, histopathological examination should be considered as an essential diagnostic tool for DFSP.

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1	Hegde U, Shetty SK, Sreeshyla HS and Doddawada VG: Dermatofibrosarcoma protuberans - a recurrent lesion with unusual presentation in the parotid region. J Clin Diag Res. 8:130–131. 2014.
2	Zheng Z, Piao J, Lee JH, Kim SE, Kim SC, Chung KY and Roh MR: Dermatofibrosarcoma protuberans: A study of clinical, pathologic, genetic, and therapeutic features in Korean patients. Yonsei Med J. 56:440–446. 2015. View Article : Google Scholar : PubMed/NCBI
3	Saiag P, Grob JJ, Lebbe C, et al: Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline. Eur J Cancer. Jul 16–2015.(Epub ahead of print). View Article : Google Scholar
4	Erdem O, Wyatt AJ, Lin E, Wang X and Prieto VG: Dermatofibrosarcoma protuberans treated with wide local excision and followed at a cancer hospital: Prognostic significance of clinicopathologic variables. Am J Dermatopathol. 34:24–34. 2012. View Article : Google Scholar : PubMed/NCBI
5	Kim M, Huh CH, Cho KH and Cho S: A study on the prognostic value of clinical and surgical features of dermatofibrosarcoma protuberans in Korean patients. J Eur Acad Dermatol Venereol. 26:964–971. 2012. View Article : Google Scholar : PubMed/NCBI
6	Suit H, Spiro I, Mankin HJ, Efird J and Rosenberg AE: Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol. 14:2365–2369. 1996.PubMed/NCBI
7	Llombart B, Serra-Guillén C, Monteagudo C, López Guerrero JA and Sanmartíin O: Dermatofibrosarcoma protuberans: A comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 30:13–28. 2013. View Article : Google Scholar : PubMed/NCBI
8	McArthur G: Molecularly targeted treatment for dermatofibrosarcoma protuberans. Semin Oncol. 31:30–36. 2004. View Article : Google Scholar : PubMed/NCBI
9	Sjöblom T, Shimizu A, O'Brien KP, Pietras K, Dal Cin P, Buchdunger E, Dumanski JP, Ostman A and Heldin CH: Growth inhibition of dermatofibrosarcoma protuberans tumors by the platelet-derived growth factor receptor antagonist STI571 through induction of apoptosis. Cancer Res. 61:5778–5783. 2001.PubMed/NCBI
10	Kiuru-Kuhlefelt S, El-Rifai W, Fanburg-Smith J, Kere J, Miettinen M and Knuutila S: Concomitant DNA copy number amplification at 17q and 22q in dermatofibrosarcoma protuberans. Cytogenet Cell Genet. 92:192–195. 2001. View Article : Google Scholar : PubMed/NCBI
11	Qiao J, Patel KU, López-Terrada D and Fang H: Atrophic dermatofibrosarcoma protuberans: Report of a case demonstrated by detecting COL1A1-PDGFB rearrangement. Diagn Pathol. 7:1662012. View Article : Google Scholar : PubMed/NCBI
12	Loss L and Zeitouni NC: Management of scalp dermatofibrosarcoma protuberans. Dermatol Surg. 31:1428–1433. 2005. View Article : Google Scholar : PubMed/NCBI
13	Garcia C, Clark RE and Buchanan M: Dermatofibrosarcoma protuberans. Int J Dermatol. 35:867–871. 1996. View Article : Google Scholar : PubMed/NCBI
14	Llombart B, Monteagudo C, Sanmartin O, López-Guerrero JA, Serra-Guillén C, Poveda A, Jorda E, Fernandez-Serra A, Pellíin A, Guillén C and Llombart-Bosch A: Dermatofibrosarcoma protuberans: A clinicopathological, immunohistochemical, genetic (COL1A1-PDGFB) and therapeutic study of low-grade versus high-grade (fibrosarcomatous) tumors. J Am Acad Dermatol. 65:564–575. 2011. View Article : Google Scholar : PubMed/NCBI
15	Maki RG, Awan RA, Dixon RH, Jhanwar S and Antonescu CR: Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. Int J Cancer. 100:623–626. 2002. View Article : Google Scholar : PubMed/NCBI
16	Hanabusa M, Kamo R, Harada T and Ishii M: Dermatofibrosarcoma protuberans with atrophic appearance at early stage of the tumor. J Dermatol. 34:336–339. 2007. View Article : Google Scholar : PubMed/NCBI
17	Martin L, Piette F, Blanc P, Mortier L, Avril MF, Delaunay MM, Dréno B, Granel F, Mantoux F, Aubin F, et al: Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans. Br J Dermatol. 153:932–936. 2005. View Article : Google Scholar : PubMed/NCBI
18	Haycox CL, Odland PB, Olbricht SM and Piepkorn M: Immunohistochemical characterization of dermatofibrosarcoma protuberans with practical applications for diagnosis and treatment. J Am Acad Dermatol. 37:438–444. 1997. View Article : Google Scholar : PubMed/NCBI
19	Dominguez-Malagón HR, Ordóňez NG and Mackay B: Dermatofibrosarcoma protuberans: Ultrastructural and immunocytochemical observations. Ultrastruct Pathol. 19:281–289. 1995. View Article : Google Scholar : PubMed/NCBI
20	Sachdev R and Sundram U: Expression of CD163 in dermatofibroma, cellular fibrous histiocytoma and dermatofibrosarcoma protuberans: Comparison with CD68, CD34 and Factor XIIIa. J Cutan Pathol. 33:353–360. 2006. View Article : Google Scholar : PubMed/NCBI
21	Bakry O and Attia A: Atrophic dermatofibrosarcoma protuberans. J Dermatol Case Rep. 6:14–17. 2012. View Article : Google Scholar : PubMed/NCBI
22	Marque M, Bessis D, Pedeutour F, Viseux V, Guillot B and Fraitag-Spinner S: Medallion-like dermal dendrocyte hamartoma: The main diagnostic pitfall is congenital atrophic dermatofibrosarcoma. Br J Dermatol. 160:190–193. 2009. View Article : Google Scholar : PubMed/NCBI
23	Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM, Brennan MF and Lewis JJ: Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution. Cancer. 88:2711–2720. 2000. View Article : Google Scholar : PubMed/NCBI
24	Wu JK, Malik MM and Egan CA: Atrophic dermatofibrosarcoma protuberans: An uncommon and misleading variant. Australas J Dermatol. 45:175–177. 2004. View Article : Google Scholar : PubMed/NCBI
25	Labropoulos SV, Fletcher JA, Oliveira AM, Papadopoulos S and Razis ED: Sustained complete remission of metastatic dermatofibrosarcoma protuberans with imatinib mesylate. Anticancer Drugs. 16:461–466. 2005. View Article : Google Scholar : PubMed/NCBI
26	Rutkowski P, Debiec-Rychter M, Nowecki Z, Michej W, Symonides M, Ptaszynski K and Ruka W: Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection. J Eur Acad Dermatol Venereol. 25:264–270. 2011. View Article : Google Scholar : PubMed/NCBI
27	Price VE, Fletcher JA, Zielenska M, Cole W, Viero S, Manson DE, Stuart M and Pappo AS: Imatinib mesylate: An attractive alternative in young children with large, surgically challenging dermatofibrosarcoma protuberans. Pediatr Blood Cancer. 44:511–515. 2005. View Article : Google Scholar : PubMed/NCBI
28	Kohli N and Srivastava D: Dermatofibrosarcoma protuberans. Dermatology Atlas for Skin of Color. Jackson-Richards D and Pandya AG: (Berlin). Springer. 269–272. 2014.