<prompt> Gene involved in Cowden syndrome isP53PTENRBRas  <response>: B PTENoPhosphatase and Tensin homolog PTEN - protein in humans encoded by the PTEN gene. Gene mutations promotes development of cancers.?Cowdens diseaseMultiple Hamartoma Syndrome:-Part of PTEN hamartoma tumor syndrome-An autosomal dominant syndrome-Trichilemmomas - Numerous tumors of hair follicles in face-Multiple hamartomatous polyps in GI tract, Lipomas, Granulomas-Very high risk of breast, Follicular endometrail carcinoma thyroid carcinomaoTreatment: Bilateral mastectomies recommended-Contraindicated are mammography other radiation exposure of breast tissue.-Mean age at presentation 10 years-Very high risk of breast, follicular carcinoma of thyroid Endometrial carcinomaoPTEN phosphatase and tensin homologue is a membrane-associated phosphatase encoded by a gene on chromosome 10q23 that is mutated in Cowden syndrome, an autosomal dominant disorder marked by frequent benign growths, such as skin appendage tumors, and an increased incidence of epithelial cancers, particularly of the breast, endometrium, and thyroid.oPTEN acts as a tumor suppressor by serving as a brake on the PI3KAKT arm of the receptor tyrosine kinase pathway.oPTEN gene function is lost in many cancers through deletion, deleterious point mutations, or epigenetic silencing.SELECTED TUMOR SUPPRESSOR GENES ASSOCIATED FAMILIAL SYNDROMES CANCERS, SORTED BY CANCER HALLMARKSGene ProteinFamilial SyndromesAssociated CancersInhibitors of Mitogenic Signaling PathwaysAPC Adenomatous polyposis coli proteinFamilial colonic polyps and carcinomasCarcinomas of stomach, colon, pancreas; melanoma. NF1 Neurofibromin-1Neurofibromatosis type 1 neurofibromas and malignant peripheral nerve sheath tumorsNeuroblastoma, juvenile myeloid leukemia. NF2 MerlinNeurofibromatosis type 2 acoustic schwannoma and meningiomaSchwannoma, meningioma. PTCH PatchedGorlin syndrome basal cell carcinoma, medulloblastoma, several benign tumorsBasal cell carcinoma, medulloblastoma. PTEN Phosphatase and tension homologueCowden syndrome variety of benign skin, GI, and CNS growths; breast, endometrial, and thyroid carcinomaDiverse cancers, particularly carcinomas and lymphoid tumors. SMAD2, SMAD4 SMAD2, SMAD4Juvenile polyposisFrequently mutated along with other components of TGFb signaling pathway in colonic pancreatic CaInhibitors of Ceil Cycle Progression. RB Retinoblastoma RB proteinFamilial retinoblastoma syndrome retinoblastoma, osteosarcoma, other sarcomasRetinoblastoma; osteosarcoma carcinomas of breast, colon, lung. CDKN2A p16INK4a pUARFFamilial melanomaPancreatic, breast, and esophageal carcinoma, melanoma, certain leukemiasInhibitors of Pro-growth Programs of Metabolism and Angiogenesis. VHL Von Hippel Lindau VHL proteinVon Hippel Lindau syndrome cerebellar hemangioblastoma, retinal angioma, renal cell carcinomaRenal cell carcinoma. STK11 Liver kinase B1 LKB1 or STK11Peutz-Jeghers syndrome GI polyps, GI cancers, pancreatic carcinoma and other carcinomasDiverse carcinomas 5-20 of cases, depending on type. SDHB, SDHD Succinate dehydrogenase complex subunits B DFamilial paraganglioma, familial pheochromocytomaParaganglioma 