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.
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Authors | D L Brockmeyer, M L Walker, G Thompson, D W Fults |
Journal | Pediatric neurosurgery
(Pediatr Neurosurg)
Vol. 26
Issue 1
Pg. 36-40
(Jan 1997)
ISSN: 1016-2291 [Print] Switzerland |
PMID | 9361116
(Publication Type: Case Reports, Journal Article)
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Chemical References |
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Topics |
- Adolescent
- Astrocytoma
(genetics, pathology, surgery)
- Brain Neoplasms
(genetics, pathology, surgery)
- Brain Stem
(pathology, surgery)
- Chromosomes, Human, Pair 17
- DNA, Neoplasm
(analysis)
- Gene Deletion
- Genes, p53
- Humans
- Magnetic Resonance Imaging
- Male
- Neoplasms, Second Primary
(genetics, pathology, surgery)
- Pinealoma
(genetics, pathology, surgery)
- Point Mutation
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