Case Reports

Aggressive desmoid fibromatosis: First case in a Rwandan child

Aimable Kanyamuhunga, Natalie McCall, Lisine Tuyisenge, Crispus Mumena, Daniela Cristina Stefan

Abstract


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.


Authors' affiliations

Aimable Kanyamuhunga, Department of Paediatrics and Child Health, Kigali University Teaching Hospital, Kigali; Faculty of Medicine, National University of Rwanda, Butare, Rwanda

Natalie McCall, Department of Paediatrics and Child Health, Kigali University Teaching Hospital, Kigali; Faculty of Medicine, National University of Rwanda, Butare, Rwanda

Lisine Tuyisenge, Department of Paediatrics and Child Health, Kigali University Teaching Hospital, Kigali; Faculty of Medicine, National University of Rwanda, Butare, Rwanda

Crispus Mumena, Kigali Health Institute, Kigali, Rwanda

Daniela Cristina Stefan, Department of Paediatrics and Child Health, Faculty of Health Sciences, Stellenbosch University, Parow, Cape Town, South Africa

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Keywords

Aggressive desmoid fibromatosis; Rwandan child

Cite this article

South African Journal of Child Health 2013;7(3):117-118. DOI:10.7196/SAJCH.593

Article History

Date submitted: 2013-02-18
Date published: 2013-08-30

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