South African Journal of Child Health
versión On-line ISSN 1999-7671
versión impresa ISSN 1994-3032
KANYAMUHUNGA, A et al. Aggressive desmoid fibromatosis: First case in a Rwandan child. S. Afr. j. child health [online]. 2013, vol.7, n.3, pp.117-119. ISSN 1999-7671.
Desmoid tumours are a rare group of locally aggressive, non-malignant tumours of fibroblastic origin that can result in significant morbidity due to local invasion. Facial involvement in children with aggressive fibromatosis is uncommon. We present the case of a 14-month-old Rwandan child with an aggressive desmoid tumour involving the left mid-facial region. The patient presented with severe stertor due to massive nasal obstruction. After intensive supportive care the diagnosis was confirmed histopathologically. Treatment consisted of eight courses of chemotherapy with vincristine, actinomycin-D and cyclophosphamide, followed by surgical removal of the remaining mass. The outcome was impressive and encouraging.