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Dermatofibrosarcoma protuberans on the leg: pearls in diagnosis and surgical management

Aileen Christine N. Barreiro, MD, FPDS, Johannes F. Dayrit, MD, FPDS, Gabriel R. Natividad, MD, FPCS

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 sarcomas.

Case:

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.

References

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A cross-sectional study of the prevalence and factors associated with self-medication among patients at the out-patient clinic of the department of dermatology in Rizal Medical Center

Dermatofibrosarcoma protuberans on the leg: pearls in diagnosis and surgical management

Aileen Christine N. Barreiro, MD, FPDS, Johannes F. Dayrit, MD, FPDS, Gabriel R. Natividad, MD, FPCS

Abstract

References

A cross-sectional study of the prevalence and factors associated with self-medication among patients at the out-patient clinic of the department of dermatology in Rizal Medical Center

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Dermatofibrosarcoma protuberans on the leg: pearls in diagnosis and surgical management