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NCI CANCERLIT^® Search: Von Hippel-Lindau Syndrome - February 2002

Imprima English

National Cancer Institute^®
Ultima Vez Modificado: 1 de febrero del 2002

Selective loss of chromosome 11 in pheochromocytomas associated with the VHL syndrome.

Childhood cerebellar hemangioblastoma does not predict germline or somatic mutations in the von Hippel-Lindau tumor suppressor gene.

Table of Contents

1
UI - 11850829
AU - Lui WO; Chen J; Glasker S; Bender BU; Madura C; Khoo SK; Kort E; Larsson
TI - C; Neumann HP; Teh BT Selective loss of chromosome 11 in pheochromocytomas associated with the VHL syndrome.
SO - Oncogene 2002 Feb 7;21(7):1117-22
AD - Department of Molecular Medicine, Karolinska Hospital, Stockholm, Sweden.
By using comparative genomic hybridization (CGH), we characterized the genetic profiles of 36 VHL-related pheochromocytomas. We then compared the results with those of sporadic and MEN 2-related pheochromocytomas. In 36 VHL-related tumors, loss of chromosome 3 and chromosome 11 were found in 34 tumors (94%) and 31 tumors (86%), respectively. There was significant concordance of deletions in chromosomes 3 and 11 (Kappa=0.64, P=0.0095), suggesting that they are involved in two different but necessary and complementary genetic pathways. The loss of chromosome 11 appeared to be specific for VHL-related pheochromocytoma as it was not present in any of the 10 VHL-related CNS hemangioblastomas studied and was significantly less common when compared with (a) sporadic pheochromocytomas from previously published results (13%; P=<0.0001), and (b) MEN 2-related pheochromocytomas from this and previously published studies (30%; P=0.0012). In summary, this is the first report of a novel consistent genetic alteration that is selected and specific for VHL-related pheochromocytoma, besides the two hits of the VHL gene.

2
UI - 11835384
AU - Fisher PG; Tontiplaphol A; Pearlman EM; Duffner PK; Hyder DJ; Stolle CA;
TI - Vortmeyer AO; Zhuang Z Childhood cerebellar hemangioblastoma does not predict germline or somatic mutations in the von Hippel-Lindau tumor suppressor gene.
SO - Ann Neurol 2002 Feb;51(2):257-60
AD - Department of Neurology, Stanford University School of Medicine, Palo Alto, CA 94305-5235, USA. pfisher@stanford.edu
Tumor suppressor gene "knockout" models would predict that children who present with hemangioblastoma are likely to harbor germline mutation of the von Hippel-Lindau gene. We screened 6 pediatric patients with cerebellar hemangioblastoma for germline or somatic mutations of the von Hippel-Lindau gene. Two had prior clinical manifestations of von Hippel-Lindau disease and, as expected, had germline von Hippel-Lindau gene mutations. Four children with solitary hemangioblastoma did not have a detectable germline deletion, rearrangement, or point mutation in their von Hippel-Lindau gene, and tumor specimens in 3 of these 4 showed no somatic von Hippel-Lindau allelic loss. Solitary cerebellar hemangioblastoma in children does not predict a germline or somatic mutation in the von Hippel-Lindau tumor suppressor gene. The tumorigenesis of hemangioblastoma in younger patients may differ from that in adults, and may involve a molecular process unrelated to the von Hippel-Lindau tumor suppressor pathway.

The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.

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