title: Astrocytoma and pineoblastoma arising sequentially in the fourth ventricle of the same patient. Case report and molecular analysis.
abstract: The sequential appearance of two different brain tumors in the same patient without intervening radiation or chemotherapy is a rare event, most often seen in hereditary cancer syndromes. We present one such case of sequential tumors, along with their molecular analysis. A 17-year-old male presented with a pilocytic astrocytoma arising in the fourth ventricle at the pontomedullary junction. Six and one half years later, a pineoblastoma was discovered in the fourth ventricle, rostral to the first tumor site. Both tumors were treated by gross-total surgical resection. Following resection of the pineoblastoma, the patient underwent craniospinal irradiation and systemic chemotherapy. Single-strand conformation polymorphism analysis showed that the patient had neither a germ-line mutation nor a somatic tumor mutation in the p53 tumor suppressor gene. Coupled with the lack of a family history of cancer, these data suggest that these were not manifestations of Li-Fraumeni syndrome, but rather two sporadic tumors which arose via a p53-independent mechanism.
authors.affiliation: Division of Pediatric Neurosurgery, Primary Children's Medical Center, Salt Lake City, Utah, USA.
authors.forename: D L
authors.initials: DL
authors.lastname: Brockmeyer
authors.forename: M L
authors.initials: ML
authors.lastname: Walker
authors.forename: G
authors.initials: G
authors.lastname: Thompson
authors.forename: D W
authors.initials: DW
authors.lastname: Fults
mesh_terms: D000293:Adolescent; D001254:Astrocytoma / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D001933:Brain Stem / Q000473:pathology* / Q000601:surgery; D002886:Chromosomes, Human, Pair 17; D004273:DNA, Neoplasm / Q000032:analysis; D017353:Gene Deletion; D016158:Genes, p53; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D016609:Neoplasms, Second Primary / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D010871:Pinealoma / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D017354:Point Mutation
chemical_list: D004273:DNA, Neoplasm