Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Ambry Genetics | RCV000491825 | SCV000580052 | pathogenic | Hereditary cancer-predisposing syndrome | 2015-02-18 | criteria provided, single submitter | clinical testing | The c.141delG pathogenic mutation, located in coding exon 2 of the PTEN gene, results from a deletion of one nucleotide at nucleotide position 141, causing a translational frameshift with a predicted alternate stop codon. This mutation has been previously identified in one kindred with multiple individuals fulfilling clinical diagnostic criteria for Cowden syndrome as well as a strong family history of malignancies, including two individuals affected with thyroid cancer (Lachlan KL, J. Med. Genet. 2007 Sep; 44(9):579-85). In addition to the clinical data presented in the literature, since frameshifts are typically deleterious in nature, this alteration is interpreted as a disease-causing mutation (ACMG Recommendations for Standards for Interpretation and Reporting of Sequence Variations. Revision 2007. Genet Med. 2008;10:294). |