Annotation Detail

Information
Associated Genes
VHL
Associated Variants
VHL p.Phe136Ser (p.F136S) ( ENST00000345392.3, ENST00000256474.3, ENST00000696143.2, ENST00000696153.1, ENST00000713811.1, ENST00000713812.1, ENST00000713815.1, ENST00000713982.1 )
VHL p.Phe136Ser (p.F136S) ( 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
Data was collected from 82 VHL mutation carriers in the Dutch VHL surveillance program. One patient was found with this missense varaint in the VHL gene. The patient was 41Y at the last follow-up and presented with: cerebellar hemangioblastoma, spinal cord hemangioblastoma, renal cell carcinoma, renal cysts and pancreatic cysts. Family history was not described.
Variant Origin
germline
Variant Origin
Rare Germline
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/6512
Gene URL
https://civic.genome.wustl.edu/links/genes/58
Variant URL
https://civic.genome.wustl.edu/links/variants/1801
Rating
3
Evidence Type
Predisposing
Disease
Von Hippel-Lindau Disease
Evidence Direction
Supports
Evidence Level
C
Clinical Significance
Uncertain Significance
Pubmed
24132471
Drugs