Dermatofibrosarcoma Protuberans on the leg: Pearls in diagnosis and surgical management
- Author:
Aileen Christine N. Barreiro
1
;
Johannes F. Dayrit
2
,
3
;
Gabriel R. Natividad
3
Author Information
- Publication Type:Other Types
- MeSH: Dermatofibrosarcoma; Neoplasms
- From: Journal of the Philippine Dermatological Society 2018;27(1):84-86
- CountryPhilippines
- Language:English
-
Abstract:
Introduction:Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue tumor accounting for less
than 0.1% of all malignant neoplasms and approximately 1% of all soft tissue sarcomas1
.
Case Summary:We report a case of a 27-year-old female who presented with a 1-year history of a gradually enlarging firm, erythematous to violaceous nodule on the right anterolateral leg. Shave excision biopsy was done, and revealed spindle-shaped fibroblasts arranged in a storiform pattern around an indistinct vasculature. Histopathologic diagnosis revealed dermatofibrosarcoma protuberans. There was prominent, diffuse, positivity for CD34 by immunohistochemistry. The cytogenetic analysis revealed a t(17;22) translocation, confirming our working impression.The patient underwent wide excision with frozen section. All surgical margins of resection were negative for tumor. The patient was referred to plastic surgery for post-excision coverage defect. Split-thickness skin graft was applied over the defect. Patient tolerated the procedure well, and has not experienced recurrence of the tumor.
Conclusion:A protuberant, well-circumscribed tumor that seems benign may incidentally be a sarcoma. Skin biopsy, immunohistochemistry and cytogenetic studies using Fluorescence In-Situ Hybridization (FISH) are the most essential laboratory investigations to validate a diagnosis of DFSP. Proper surgical excision with adequate margins will prevent recurrence of the tumor. - Full text:84-86 What-lies-beneath_DFSP_Barreiro.pdf
