Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clingen PTEN Variant Curation Expert Panel, |
RCV000988410 | SCV002576574 | uncertain significance | PTEN hamartoma tumor syndrome | 2020-10-20 | reviewed by expert panel | curation | PTEN c.-802G>A, also described as c.-801G>A (NC_000010.11:g.87863668G>A) is currently classified as a variant of uncertain significance for PTEN Hamartoma Tumor syndrome in an autosomal dominant manner using modified ACMG criteria (PMID 30311380). Please see a summary of the rules and criteria codes in the “PTEN ACMG Specifications Summary” document (assertion method column). PM2: Absent in large sequenced populations |
Gene |
RCV000222615 | SCV000279700 | uncertain significance | not specified | 2015-12-17 | criteria provided, single submitter | clinical testing | This variant is denoted PTEN c.-802G>A, and describes a nucleotide substitution 802 base pairs upstream of the ATG translational start site in the PTEN core promoter region. The surrounding sequence, with the base that is substituted in braces, is GAGA{G/A}TTGA. This variant, also called c.-801G>A using alternate numbering, has not been published in the literature to our knowledge. This variant occurs within a region of the PTEN promoter (c.-798 to c.-1238) in which variants have been observed in individuals with features of Cowden syndrome (Zhou 2003). Based on currently available information, it is unclear whether PTEN c.-802G>A is pathogenic or benign. We consider it to be a variant of uncertain significance. |
Mendelics | RCV000988410 | SCV001138119 | likely benign | PTEN hamartoma tumor syndrome | 2019-05-28 | criteria provided, single submitter | clinical testing |