Dermatofibrosarcoma protuberans in children: an update on the diagnosis and treatment.

摘要: Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of low grade malignant potential. Although rare, pediatric cases pose particular challenge in diagnosis and management. In children, the clinical appearance may be heterogeneous high index suspicion necessary to avoid delays which can lead further morbidity. Histologic examination, often with use appropriate immunostains, for diagnosis. Advances understanding molecular genetics DFSP have led diagnostic therapeutic modalities. thought result from translocation between platelet-derived growth factor beta (PDGFB, 22q13.1) type 1 collagen (COL1A1, 17q21≈22) leading fusion protein (PDGFB) stimulates PDGF receptor. Detection this tissue via PCR or fluorescence situ hybridization (FISH) helpful difficult cases. While surgery wide local excision Mohs micrographic mainstay treatment, targeted therapy imatanib mesylate shows promise large unresectable tumors. Knowledge features, histology, genetics, treatment options important successful management these