title: Long-term survival in a child with a central nervous system medulloepithelioma.
abstract: Central nervous system medulloepitheliomas are extremely rare and malignant (World Health Organization Grade IV) primitive neuroectodermal tumors (PNETs) that arise in childhood. Unlike other PNETs, medulloepitheliomas have a dismal prognosis, with only 2 reported cases in the literature in which the patient survived beyond 5 years after treatment. The authors report on the third known case of a child who survived longer than 5 years. A review of all the published cases of medulloepithelioma is also presented, and alternative treatment strategies for PNET tumors, including high-dose chemotherapy with stem-cell rescue, are discussed.
authors.affiliation: Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California 90048, USA.
authors.forename: Parham
authors.initials: P
authors.lastname: Moftakhar
authors.forename: Xuemo
authors.initials: X
authors.lastname: Fan
authors.forename: Carole H
authors.initials: CH
authors.lastname: Hurvitz
authors.forename: Keith L
authors.initials: KL
authors.lastname: Black
authors.forename: Moise
authors.initials: M
authors.lastname: Danielpour
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D002648:Child; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D018242:Neuroectodermal Tumors, Primitive / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D015996:Survival Rate
chemical_list: D000970:Antineoplastic Agents