Infantile myofibromatosis of the central nervous system

BACKGROUND: Infantile myofibromatosis is the most common fibrous disorder of infancy and childhood. It may occur in two distinct forms: multicentric and solitary. In both cases involvement of the central nervous system (CNS) is unusual: brain myofibromas are usually intracranial in proximity of the dura mater, with infiltration of the calvarial bones and secondary brain compression. Spine myofibromas are exceptional and most of the cases reported in the literature represent secondary locations of visceral lesions. The natural history of myofibromas of the CNS in infantile myofibromatosis is characterized by a period of rapid growth, subsequent stabilization, and spontaneous regression in many cases. On these grounds surgical treatment is reserved for lesions that compress the brain or spinal cord and show an obvious progression. Small lesions or tumor residuals in asymptomatic children without visceral involvement should be closely observed as first choice. CASE REPORT: We report the case of a 10-month-old child with a solitary intramedullary myofibroma, extended from C7 to the conus. The patient underwent partial surgical removal of this lesion. Control MRI has shown the spontaneous slow reduction of the tumor residual and a progressive improvement of preoperative neurological conditions has been observed. To our knowledge this is the first case in which a solitary myofibroma was localized within the spinal cord.