title: Intraventricular rhabdoid tumor.
abstract: Malignant rhabdoid tumor (MRT) most commonly occurs in kidney. In the central nervous system, cerebellum is the most common site of occurrence. CNS rhabdoid tumors typically occur in small children, do not respond favorably to treatment and are usually fatal within 1-year. Here is reported a 4-year-old child who presented with features of raised intracranial pressure. Apart from papillodema, there were no neurological signs. Imaging revealed a left lateral ventricular heterogeneous mass abutting the foramen of monro, with mild irregular contrast enhancement and hydrocephalus. The child underwent right ventriculo-peritoneal shunt followed by craniotomy and gross total tumor resection. He was discharged 10-days after surgery without any neurological deficits. Histopathology revealed features compatible with rhabdoid tumor. Despite radiotherapy and chemotherapy, the child died of progressive disease 10-months after surgery. The highly malignant nature of this tumor makes early diagnosis essential for aggressive management and prognostication.
authors.affiliation: Department of Neurosurgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
authors.forename: Faiz Uddin
authors.initials: FU
authors.lastname: Ahmad
authors.forename: Ashish
authors.initials: A
authors.lastname: Suri
authors.forename: Ashok Kumar
authors.initials: AK
authors.lastname: Mahapatra
authors.forename: Veer Singh
authors.initials: VS
authors.lastname: Mehta
authors.forename: Ajay
authors.initials: A
authors.lastname: Garg
authors.forename: M C
authors.initials: MC
authors.lastname: Sharma
authors.forename: Epari
authors.initials: E
authors.lastname: Sridhar
mesh_terms: D002551:Cerebral Ventricle Neoplasms / Q000175:diagnosis* / Q000628:therapy; D002675:Child, Preschool; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D018335:Rhabdoid Tumor / Q000175:diagnosis* / Q000628:therapy