INFANTILE MYOFIBROMATOSIS - A CASE WITH UNUSUAL FEATURES AND REVIEW OF THE LITERATURE

A three-month-old boy was admitted for a slowly progressing nodule in his right chin, first recognized at birth and recently accompanied by facial paralysis. It was found to be a soft tissue mass arising in sub cutaneous tissue and extending deep into the temporal muscle, causing temporal bone erosion without infiltrating the dura. Initially interpr eted as mesenchymal chondrosarcoma, combined chemotherapy (PULSE-VAC) was given. Eighteen months later an additional nodule developed in the paraspinal skin. Evaluation of both lesions showed vimentin and a-smo oth-muscle-action positivity. As the final diagnosis was multicentric infantile myofibromatosis, the child was followed up without therapy. Thirty months later, multiple osteolytic lesions appeared on the skull and long bones of the extremities. Some lesions remained stable and s ome regressed during two years of follow-up.