Annotation Detail

Information
Associated Genes
VHL
Associated Variants
VHL p.? (p.?) ( ENST00000256474.3, ENST00000345392.3, ENST00000696143.2, ENST00000696153.1, ENST00000713811.1, ENST00000713812.1, ENST00000713815.1, ENST00000713982.1 )
VHL p.? (p.?) ( ENST00000256474.3, ENST00000345392.3, ENST00000696143.2, ENST00000696153.1, ENST00000713811.1, ENST00000713812.1, ENST00000713815.1, ENST00000713982.1 )
Associated Disease
von Hippel-Lindau disease
Source Database
CIViC Evidence
Description
Mutation detected in one case. Case shows clear-cell renal cell carcinoma. This case also harbored a second somatic variant in VHL: 209-210delAG (Stop130). Since this was a case of sporadic RCC, with a somatic variant observed in the tumor, we can only infer that the variant is functional and would be predisposing if it occurred in a germline context.
Variant Origin
somatic
Variant Origin
Somatic
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/1947
Gene URL
https://civic.genome.wustl.edu/links/genes/58
Variant URL
https://civic.genome.wustl.edu/links/variants/848
Rating
2
Evidence Type
Predisposing
Disease
Von Hippel-Lindau Disease
Evidence Direction
Supports
Evidence Level
E
Clinical Significance
Uncertain Significance
Pubmed
11505222
Drugs