Korean Journal of Head & Neck Oncology (대한두경부종양학회지)

The Korean Society for Head and Neck Oncology (대한두경부종양학회)
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Dermatofibrosarcoma Protuberans on the Occipital Scalp Showed Uncommon Presentation: A Case Report

비전형적 임상양상을 보이는 후두부의 융기성 피부 섬유 육종에 대한 증례보고

  • Jiwon Jeong (Department of Plastic and Reconstructive Surgery, Hallym University Kangdong Sacred Heart Hospital ) ;
  • Chul Hoon Chung (Department of Plastic and Reconstructive Surgery, Hallym University Kangdong Sacred Heart Hospital ) ;
  • SeongJin Cho (Department of Pathology, Hallym University Kangdong Sacred Heart Hospital)
  • 정지원 (한림대학교 강동성심병원 성형외과) ;
  • 정철훈 (한림대학교 강동성심병원 성형외과) ;
  • 조성진 (한림대학교 강동성심병원 병리과)
  • Received : 2023.12.07
  • Accepted : 2024.01.24
  • Published : 2024.05.30
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Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma, with an incidence of about 0.8% to 5% per million people per year, accounting for 1% of soft tissue sarcomas. In its early stage, DFSP is typically found as a violet or pinkish macule or patch, and it can develop into a palpable mass with ulceration or bleeding. The standard treatment for DFSP is wide local excision of the tumor with a 2- to 3-cm negative margin, and radiation therapy or chemotherapy can be conducted with surgical treatment. A 35-year-old man had a palpable mass on the left side of his occipital scalp without color change, ulceration, or bleeding, which typically are present in malignancy. A magnetic resonance imaging (MRI) scan showed a 3-cm homogenous enhanced mass without adhesion between the scalp and the mass. Unexpectedly, a biopsy revealed the round mass to be DFSP. A wide excision and rotation of the scalp flap were performed. The patient recovered without any complications and received adjuvant radiotherapy at a dose of 60 Gray (Gy) for six weeks. There was no recurrence through six months of follow-up. Here we report this unique case of DFSP with atypical presentation.

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References

  1. Mujtaba B, Wang F, Taher A, Aslam R, Madewell JE, Spear R, et al. Dermatofibrosarcoma protuberans: Pathological and imaging review. Curr Probl Diagn Radiol. 2021;50:236-240.  https://doi.org/10.1067/j.cpradiol.2020.05.011
  2. Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer. 2004;101:2503-2508.  https://doi.org/10.1002/cncr.20678
  3. Luu C, Messina JL, Brohl AS, Sondak VK. Dermatofibrosarcoma protuberans. In: Reghavan D, Ahluwalia MS, Blanke CD, Brown J, Kim ES, Reaman GH, Sekeres MA, editors. Textbook of uncommon cancer. 5th ed. Wiley-Blackwell; 2017. p.994-1001. 
  4. Allen A, Ahn C, Sangueza OP. Dermatofibrosarcoma protuberans. Dermatol Clin. 2019;37:483-488.  https://doi.org/10.1016/j.det.2019.05.006
  5. Hoffman E. Uber das knollentreibende fibrosarkom der haut (Dermatofibrosarkoma protuberans). Dermatol. 1925;43:1-28.  https://doi.org/10.1159/000250699
  6. Kampshoff JL, Cogbill TH. Unusual skin tumors: Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans. Surg Clin North Am. 2009;89:727-738.  https://doi.org/10.1016/j.suc.2009.02.005
  7. Paradisi A, Abeni D, Rusciani A, Cigna E, Wolter M, Scuderi N, et al. Dermatofibrosarcoma protuberans: Wide local excision vs. Mohs micrographic surgery. Cancer Treat Rev. 2008;34:728-736.  https://doi.org/10.1016/j.ctrv.2008.06.002
  8. Weinstein JM. Congenital dermatofibrosarcoma protuberans. Arch Dermatol. 2003;139:207-211.  https://doi.org/10.1001/archderm.139.2.207
  9. Checketts SR, Hamilton TK, Baughman RD. Congenital and childhood dermatofibrosarcoma protuberans: A case report and review of the literature. J Am Acad Dermatol. 2000;42:907-913.  https://doi.org/10.1016/S0190-9622(00)90270-6
  10. Thway K, Noujaim J, Jones RL, Fisher C. Dermatofibrosarcoma protuberans: Pathology, genetics, and potential therapeutic strategies. Ann Diagn Pathol. 2016;25:64-71.  https://doi.org/10.1016/j.anndiagpath.2016.09.013
  11. Nakamura I, Kariya Y, Okada E, Yasuda M, Matori S, Ishikawa O, et al. A novel chromosomal translocation associated with COL1A2-PDGFB gene fusion in dermatofibrosarcoma protuberans: PDGF expression as a new diagnostic tool. JAMA Dermatol. 2015;151:1330-1337.  https://doi.org/10.1001/jamadermatol.2015.2389
  12. Bogucki B, Neuhaus I, Hurst EA. Dermatofibrosarcoma protuberans: A review of the literature. Dermatol Surg. 2012;38:537-551.  https://doi.org/10.1111/j.1524-4725.2011.02292.x
  13. Jang HU, Chou YW. Scalp reconstruction: a 10-year experience. Arch Craniofac Surg 2020;21:237-243. https://doi.org/10.7181/acfs.2020.00269