Issue N# 3 - 2006
PAEDIATRICS
Giant form of infantile myofibromatosis located on the jaw
Authors : A. Benhammou, M. Boujemaoui, R. Bencheikh, M. A. Benbouzid, M. Boulaich, L. Essakali, M. Kzadri, M. Benhammou (Rabat)
Ref. : Rev Laryngol Otol Rhinol. 2006;127,3:171-174.
Article published in french 
Downloadable PDF document french
Summary :
Introduction: Infantile myofibromatosis is a rare fibrovascular-like tumour, characterized by the development of single or multiple nodular lesions arising from cutaneous, subcutaneous, muscular, bone or visceral structures, diagnosed before 2 years. Observation: We report a case of infantile myofibromatosis located on the jaw, which is unique because of its large size (12 cm), its location and its neonatal presentation. It was a voluminous proliferate tumour with an ulcerated centre, located on the left jaw. Surgical excision was complete and the diagnosis was maded on histological examination. Recovery was uncomplicated with no recurrence on follow up. Discussion: Diagnosis of infantile myofibromatosis is difficult because of the clinical heterogeneity and the histopathological appearance. The histological diagnosis relies on identification of two separate components, fascicular myofibroblastic at the periphery and hemangiopericytome in the centre. The most frequent treatment is conservative surgical excision, because recurrence rates are low and there is a possibility of spontaneous regression. Some authors recommend conservative management of very large or multiple lesions particularly if excision will result in significant functional or cosmetic morbidity.
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