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[Childhood-onset multifocal atrophic dermatofibrosarcoma].

E Kostrzewa¹, M Beylot-Barry, B Vergier

¹Service de Dermatologie, Hôpital Haut Lévêque, CHU de Bordeaux.

Annales De Dermatologie Et De Venereologie

|May 31, 2006

Summary

Atrophic dermatofibrosarcoma protuberans, a rare childhood tumor, is often diagnosed late due to its slow growth and benign appearance. Early diagnosis and characteristic genetic markers like t(17;22) are crucial for effective treatment.

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Area of Science:

Dermatopathology
Pediatric Oncology
Medical Genetics

Background:

Atrophic dermatofibrosarcoma protuberans (DFSP) is a rare variant of DFSP, typically seen in children and young adults.
This variant often presents with subtle clinical features, making early diagnosis challenging.

Observation:

A case of multifocal atrophic DFSP with childhood onset is presented, diagnosed at age 29.
The lesion was a large, asymptomatic macular brown plaque on the thigh, initially treated with limited excision.

Findings:

Diagnosis of childhood atrophic DFSP is frequently delayed due to slow progression, asymptomatic nature, and benign appearance.
Histopathology, immunohistochemistry, and cytogenetic studies detecting the characteristic t(17;22) translocation are vital for confirming DFSP diagnosis.

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Implications:

Timely diagnosis of atrophic DFSP, particularly in pediatric cases, is essential for appropriate management.
Standard treatment involves extensive surgical excision, with Mohs surgery for specific cases and imatinib for metastatic disease.