Multicentrische infantiele myofibromatose

A 7 days old baby presented with a tumour in the face. Additional investigations revealed multicentric myofibromatosis in the face, liver and middle ear, later on complicated by right sided peripheral facial nerve palsy. Myofibromatosis is a rare condition. It is however the most common fibrous tumour of infancy. Histopathology is necessary for a definitive diagnosis. This should be done in a tertiary centre, possibly preceded by an MRI. It is important to give the parents certainty about diagnosis and prognosis. Extra cutaneous myofibroma and/or visceral involvement can be demonstrated/excluded by performing additional imaging. Most myofibroma regress spontaneously and treatment is conservative. Prognosis is poor for infants with visceral involvement. In this patient chemotherapy for one year was chosen because of the visceral involvement and rapid progression of the myofibroma with compression of the facial nerve. After one year of chemotherapy the myofibroma in the liver and face almost disappeared but the myofibroma, located at the ganglion geniculi, remained unchanged with persisting right sided facial nerve palsy.