Dermatofibrosarcoma protuberans is a rare low-grade sarcoma, which rarely metastasizes, but it is locally aggressive with a propensity to recur. It usually affects persons of African descent and is extremely rare in childhood with a favourable prognosis. We present a case of paediatric dermatofibrosarcoma protuberans to the midline of the lower back of a 9-year-old Afro-Caribbean boy who was biopsied with a 2-mm margin. After histological confirmation, a 4-cm margin was then performed. Surveillance for recurrence, though none has been seen thus far after 6-month follow-up, will be done for at least 5 years and possibly longer, given this is the first case of this nature ever seen in our institution and the Caribbean region.
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http://dx.doi.org/10.1093/jscr/rjae062 | DOI Listing |
Indian Dermatol Online J
May 2024
Department of Pathology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.
Clin Radiol
December 2024
Barts Health NHS Trust W Smithfield, London, EC1A 7BE, UK.
Glob Pediatr Health
September 2024
Pediatric Plastic surgery Unit, Children's Hospital of Rabat, Morocco.
Mod Pathol
September 2024
Diagnostic Laboratory, Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands; Department of Pathology, Radboud University Medical Center, Nijmegen, The Netherlands.
EGFR aberrations are reported in a subset of myofibroblastic lesions with kinase domain duplication (EGFR-KDD) and exon 20 mutations being assigned to infantile fibrosarcomas (IFS), mesoblastic nephroma, and fibrous hamartoma of infancy (FHI), respectively. In this retrospective study, we correlated molecular findings with the histomorphology of 14 myofibroblastic lesions harboring such genetic changes identified by NGS. We additionally performed DNA methylation profiling (DNAmp) and immunohistochemistry.
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