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Dermatofibrosarcoma protuberans, fibrosarcomatous variant: A rare tumor in children
Author(s) -
Chicaud Matthieu,
FrassatiBiaggi Anciade,
Kaltenbach Sophie,
Karanian Marie,
Orbach Daniel,
Fraitag Sylvie
Publication year - 2020
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.14393
Subject(s) - dermatofibrosarcoma protuberans , medicine , fluorescence in situ hybridization , young adult , dermatology , malignant transformation , pathology , chemistry , biochemistry , chromosome , gene
Abstract Fibrosarcomatous transformation of dermatofibrosarcoma protuberans is associated with a significantly worse prognosis in adults, but is a very rare feature in the pediatric population. Here, we report a case that occurred in a child. The diagnosis of fibrosarcomatous transformation of dermatofibrosarcoma protuberans was confirmed by a histopathological assessment and fluorescence in situ hybridization. A comparison with eleven other patients reported in the literature revealed that the local recurrence and mortality rates in children are similar to those observed in adults.