Total submissions: 5
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clingen PTEN Variant Curation Expert Panel, |
RCV002286568 | SCV002576573 | uncertain significance | PTEN hamartoma tumor syndrome | 2020-10-20 | reviewed by expert panel | curation | PTEN c.-943C>T (NC_000010.10:g.89623283C>T) 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). No criteria currently apply to this variant. |
Gene |
RCV000258977 | SCV000149486 | uncertain significance | not provided | 2024-02-05 | criteria provided, single submitter | clinical testing | Has not been previously published as pathogenic or benign to our knowledge; Not observed at significant frequency in large population cohorts (gnomAD); Also known as c.-943C>T |
Ambry Genetics | RCV000115577 | SCV000187185 | uncertain significance | Hereditary cancer-predisposing syndrome | 2014-03-25 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV000763681 | SCV000894561 | uncertain significance | Macrocephaly-autism syndrome; Familial meningioma; Malignant tumor of prostate; VACTERL with hydrocephalus; Glioma susceptibility 2; PTEN hamartoma tumor syndrome; Cowden syndrome 1 | 2018-10-31 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV001175018 | SCV001338534 | likely benign | not specified | 2024-09-09 | criteria provided, single submitter | clinical testing | Variant summary: PTEN c.-944C>T is located in the untranscribed region upstream of the PTEN gene region. The variant allele was found at a frequency of 6e-05 in 383106 control chromosomes, predominantly at a frequency of 9.2e-05 within the Non-Finnish European subpopulation in the gnomAD database (v4.1 dataset). The observed variant frequency within Non-Finnish European control individuals in the gnomAD database is approximately 5.9-fold of the estimated maximal expected allele frequency for a pathogenic variant in PTEN causing Hereditary Breast And Ovarian Cancer Syndrome phenotype (1.6e-05). The variant, c.-944C>T, has been reported in the literature in an individual affected with breast- and/or ovarian cancer (Guglielmi_2021), and although patients I this cohort had positive family history for the disease, no supporting evidence for causality for this variant was provided. This report does not provide unequivocal conclusions about association of the variant with Hereditary Breast And Ovarian Cancer Syndrome. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. The following publication have been ascertained in the context of this evaluation (PMID: 34299313). ClinVar contains an entry for this variant (Variation ID: 127682). Based on the evidence outlined above, the variant was classified as likely benign. |