Primary meningeal rhabdomyosarcoma in a child with hypomelanosis of Ito

Intracranial rhabdomyosarcomas are rare neoplasms, and those thought to be primary meningeal tumors are even more rare. Hypomelanosis of Ito is a neur ocutaneous disorder believed to involve a defect in cells of neural crest o rigin. We report the case of a 15-month-old boy with hypomelanosis of Ito w ho developed a primary meningeal rhabdomyosarcoma. The patient initially pr esented with hydrocephalus and 2 months later developed neurologic signs lo calizing to the spinal cord. Radiologic studies revealed widespread leptome ningeal enhancement with compression of the spinal cord at C5-C7. A brain b iopsy revealed a tumor diffusely involving the meninges. Microscopically, t he tumor was composed of rhabdomyoblasts, many of which showed prominent cr oss-striations on routine hematoxylin-eosin staining. To the best of our kn owledge, this is the first reported case of meningeal rhabdomyosarcoma in a patient with hypomelanosis of Ito and the fourth reported case of a primar y meningeal rhabdomyosarcoma reported in the world literature.