DERMATOFIBROSARCOMA PROTUBERANS IN PEDIATRIC AGE

  • Victoria Guiote Assistente Hospitalar de Dermatologia e Venereologia/Consultant, Dermatology and Venereology, Serviço de Dermatologia do Centro Hospitalar Leiria, Portugal
  • Ricardo Vieira Assistente Hospitalar de Dermatologia/Consultant, Dermatology and Venereology, Centro Hospitalar e Universitário de Coimbra, Portugal
  • Fernanda Cunha Assistente Graduada de Anatomia Patológica/Graduated Consultant, Pathology, Centro Hospitalar Leiria, Portugal
Keywords: Dermatofibrosarcoma, Skin neoplasms, child, Imatinib, mesylate

Abstract

Dermatofibrosarcoma protuberans is a rare fibrohistiocytic tumor with frequent locoregional recurrences and a low metastatic ability. Only 6% of dermatofibrosarcoma protuberans are diagnosed in children. The gold standard treatment is the surgical excision and the lower recurrence rates are observed with wide surgical margins (2,5cm) or with margin control by Mohs micrographic surgery. The authors report the case of a female 8-year-old child with a dermatofibrosarcoma protuberans of the abdominal wall. The tumor was excised with wide margins with a good outcome after a 12-month follow-up. The typical features of this tumor in children and the main differential diagnoses were pointed out.

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How to Cite
Guiote, V., Vieira, R., & Cunha, F. (1). DERMATOFIBROSARCOMA PROTUBERANS IN PEDIATRIC AGE. Journal of the Portuguese Society of Dermatology and Venereology, 72(3), 377-381. https://doi.org/10.29021/spdv.72.3.282
Section
Case Reports