Dermatofibrosarcoma Protuberans with Fibrosarcomatous Change: A Case Report of Rare Entity

Authors

  • Chinathip Theerawattanawit Division of Dermatology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
  • Rachot Wongjirattikarn Division of Dermatology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
  • Jade Wititsuwannakul Division of Dermatology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

Keywords:

Dermatofibrosarcoma protuberans, Dermatofibrosarcoma protuberans with fibrosarcomatous changes

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare malignancy affecting the skin and underlying soft tissues. Typically characterized by a favorable prognosis with low metastatic and recurrence rates, DFSP can manifest as a subtype with an unfavorable outcome known as DFSP with fibrosarcomatous changes (DFSP-FS). Histologic examination reveals DFSP-FS to exhibit more atypical cellular features and increased mitotic activity compared to the classic DFSP variant. In this report, we detail the case of a 48-year-old Thai female patient presenting with a progressively enlarging mass on the lumbosacral area. Pathological and immunohistochemical analyses confirmed the diagnosis of DFSP-FS. This case presents the rarity of DFSP-FS and emphasizes its distinctive pathological and immunohistochemical characteristics.

References

Monnier D, Vidal C, Martin L, et al. Dermatofibrosarcoma protuberans: a population-based cancer registry descriptive study of 66 consecutive cases diagnosed between 1982 and 2002. J Eur Acad Dermatol Venereol 2006;20:1237-42.

Brooks J, Ramsey ML. Dermatofibrosarcoma Protuberans. 2023 Apr 14. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024. PMID: 30020677.

McArthur GA, Demetri GD, Oosterom Av, et al. Molecular and Clinical Analysis of Locally Advanced Dermatofibrosarcoma Protuberans Treated With Imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol 2005;23:866-73.

Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD. A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change. J Am Acad Dermatol 2014;71:781-6.

Reha J, Katz SC. Dermatofibrosarcoma Protuberans. Surg Clin North Am 2016;96:1031-46.

Voth H, Landsberg J, Hinz T, et al. Management of dermatofibrosarcoma protuberans with fibrosarcomatous transformation: an evidence-based review of the literature. J Eur Acad Dermatol Venereol 2011;25:1385-91.

Erdem O, Wyatt AJ, Lin E, Wang X, Prieto VG. Dermatofibrosarcoma Protuberans Treated With Wide Local Excision and Followed at a Cancer Hospital: Prognostic Significance of Clinicopathologic Variables. Am J Dermatopathol 2012;34:24-34.

Abbott JJ, Oliveira AM, Nascimento AG. The Prognostic Significance of Fibrosarcomatous Transformation in Dermatofibrosarcoma Protuberans. Am J Surg Pathol 2006;30:436-43.

Rutkowski P, Dębiec-Rychter M, Nowecki Z, Michej W, Symonides M, Ptaszynski K, et al. Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection. J Eur Acad Dermatol Venereol 2011;25:264-70.

Abbott JJ, Erickson-Johnson M, Wang X, Nascimento AG, Oliveira AM. Gains of COL1A1-PDGFB genomic copies occur in fibrosarcomatous transformation of dermatofibrosarcoma protuberans. Mod Pathol 2006;19:1512-8.

Downloads

Published

2024-06-05

How to Cite

Theerawattanawit, C., Wongjirattikarn, R., & Wititsuwannakul, J. (2024). Dermatofibrosarcoma Protuberans with Fibrosarcomatous Change: A Case Report of Rare Entity. Thai Journal of Dermatology, 40(2), 40–44. Retrieved from https://he02.tci-thaijo.org/index.php/TJD/article/view/267009

Issue

Section

Case Report