n South African Journal of Child Health - Aggressive desmoid fibromatosis : first case in a Rwandan child : case report
|Article Title||Aggressive desmoid fibromatosis : first case in a Rwandan child : case report|
|© Publisher:||Health and Medical Publishing Group (HMPG)|
|Journal||South African Journal of Child Health|
|Affiliations||1 Kigali University Teaching Hospital, Rwanda, 2 Kigali University Teaching Hospital, Rwanda, 3 Kigali University Teaching Hospital, Rwanda, 4 National University of Rwanda, 5 National University of Rwanda, 6 National University of Rwanda, 7 Kigali Health Institute, Rwanda and 8 Stellenbosch University|
|Publication Date||Sep 2013|
|Pages||117 - 118|
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.
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