Resumen

DOMENECH, Ana et al. Dermatofribosarcoma protuberans in pediatric patients. Cir. plást. iberolatinoam. [online]. 2016, vol.42, n.1, pp.41-46. ISSN 1989-2055.

Background and Objectives. We present 3 cases of dermato fibro sarcoma protuberans (DFSP) to document the unusual clinical presentation of this condition in pediatric patients. Patients and Methods. We conduct a retrospective descriptive analysis of the last 3 cases of DFSP treated in our institution during years 2010-2013. Results. A 12-year-old girl referred from another institution for DFSP in distal thigh; we practised surgical margins extended 3 cm resection and cover with artificial dermis and autologous graft; after 1 year follow up without recurrence, reconstruction of the defect was performed using autologous fat grafts. Another 12-year-old girl diagnosed of Multiple Hamartoma Syndrome associated with PTEN gene, who presented DFSP at the right labia major and at the right breast, being recurrent in this last location. Finally, a congenital DFSP in a patient referred at 3 months with giant tumour of abdominal wall. The initial diagnosis was made by MRI with gadolinium. The final diagnosis of DFSP was made based on histopathological findings of the piece following surgical resection, because percutaneous biopsy or skin biopsy couldn't confirm the diagnostic. Conclusions. DFSP is a rare type of sarcoma of the skin in children and adults, locally aggressive, with a high rate of recurrence. Wide local excision may cause maiming and disfiguring to the patient, thereby Mohs micrographic surgery (CMM) enables to reduce surgical margins.

Palabras clave : Dermatofibrosarcoma; Dermatofibrosarcoma protuberans; Mohs' micrographic surgery; Pediatric patients.

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