title: Primary meningeal rhabdomyosarcoma in a child with hypomelanosis of Ito.
abstract: Intracranial rhabdomyosarcomas are rare neoplasms, and those thought to be primary meningeal tumors are even more rare. Hypomelanosis of Ito is a neurocutaneous disorder believed to involve a defect in cells of neural crest origin. We report the case of a 15-month-old boy with hypomelanosis of Ito who developed a primary meningeal rhabdomyosarcoma. The patient initially presented with hydrocephalus and 2 months later developed neurologic signs localizing to the spinal cord. Radiologic studies revealed widespread leptomeningeal enhancement with compression of the spinal cord at C5-C7. A brain biopsy revealed a tumor diffusely involving the meninges. Microscopically, the tumor was composed of rhabdomyoblasts, many of which showed prominent cross-striations on routine hematoxylin-eosin staining. To the best of our knowledge, this is the first reported case of meningeal rhabdomyosarcoma in a patient with hypomelanosis of Ito and the fourth reported case of a primary meningeal rhabdomyosarcoma reported in the world literature.
authors.affiliation: Department of Pathology, East Carolina University School of Medicine, Greenville, NC 27858, USA.
authors.forename: F
authors.initials: F
authors.lastname: Xu
authors.forename: L E
authors.initials: LE
authors.lastname: De Las Casas
authors.forename: L J
authors.initials: LJ
authors.lastname: Dobbs
mesh_terms: D000199:Actins / Q000378:metabolism; D001706:Biopsy; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology; D007150:Immunohistochemistry; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008577:Meningeal Neoplasms / Q000150:complications / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D010859:Pigmentation Disorders / Q000150:complications* / Q000175:diagnosis; D012208:Rhabdomyosarcoma / Q000150:complications / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D014746:Vimentin / Q000378:metabolism
chemical_list: D000199:Actins; D014746:Vimentin