Multicentric infantile myofibromatosis

Infantile myofibromatosis (IM) is a rare tumor of infancy and childhood, typically presenting as a firm, nodular mass involving soft tissue, bone or viscera. Approximately one-third of cases involve the head and neck. These tumors can be solitary or multicentric. Biopsy reveals tumor cells that resemble myofibroblasts. Spontaneous regression may occur. A high degree of suspicion is necessary to differentiate this entity from other more aggressive processes histiocytosis, fibrosarcoma, rhabdomyosarcoma. We describe the case of a male infant with multicentric myofibromatosis, presenting with multiple thoraco-abdominal subcutaneous nodules and lytic mass lesions of the temporal bone and calvarium. The characteristic clinical, radiologic and histopathologic features of this process are reviewed along with diagnostic and therapeutic options.