South African Journal of Child Health

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Aggressive desmoid fibromatosis: First case in a Rwandan child

A Kanyamuhunga, N McCall, L Tuyisenge, C Mumena, DC Stefan


Desmoid tumours are a rare group of locally aggressive, non-malignant tumours of broblastic origin that can result in signicant morbidity due to local invasion. Facial involvement in children with aggressive bromatosis is  uncommon. We present the case of a 14-month-old Rwandan child with an aggressive desmoid tumour involving the le mid-facial region. e patient presented with severe stertor due to massive nasal obstruction. Aer intensive supportive care the diagnosis was conrmed histopathologically. Treatment consisted of eight courses of chemotherapy with vincristine, actinomycin-D and cyclophosphamide, followed by surgical removal of the remaining mass. e
outcome was impressive and encouraging.
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