title: Diffuse central neurocytoma with craniospinal dissemination.
abstract: Central neurocytomas (CN) are benign central nervous system (CNS) tumors of neuroglial origin that represent 0.25 to 0.5% of all intracranial tumors in adults and an even smaller proportion of pediatric CNS tumors. These tumors characteristically occur in the subependymal layer of the lateral ventricle near the foramen of Monro and appear as sharply demarcated, solitary lesions. Surgical resection is considered curative, as the reported recurrence rate is less than 5% for patients with localized disease. In this report, we describe the case of a three-year-old boy with a diffuse CN with craniospinal dissemination identified at the time of diagnosis. Given the extensive nature of the disease, surgical resection was not indicated and he underwent a chemotherapeutic regimen of vincristine and carboplatin. At 18 months followup, the patient has completed 6 of 8 total cycles of vincristine and carboplatin and serial imaging shows stable disease within the craniospinal axis.
authors.affiliation: Department of Neurosurgery, Massachusetts General Hospital and Harvard Medical School, 55 Fruit Street, White Building Room 502, Boston, MA, USA.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Stapleton
authors.forename: Brian P
authors.initials: BP
authors.lastname: Walcott
authors.forename: Kristopher T
authors.initials: KT
authors.lastname: Kahle
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Codd
authors.forename: Brian V
authors.initials: BV
authors.lastname: Nahed
authors.forename: Li
authors.initials: L
authors.lastname: Chen
authors.forename: Nathan J
authors.initials: NJ
authors.lastname: Robison
authors.forename: Ivana
authors.initials: I
authors.lastname: Delalle
authors.forename: Liliana C
authors.initials: LC
authors.lastname: Goumnerova
authors.forename: Eric M
authors.initials: EM
authors.lastname: Jackson
mesh_terms: D001932:Brain Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D002551:Cerebral Ventricle Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D002675:Child, Preschool; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D018306:Neurocytoma / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*