Cancer Syndromes for ABGC Boards (100% Accurate)

HBOC correct answers breast, ovarian, prostate, melanoma Familial Adenomatous Polyposis correct answers CHRPE (congenital hypertrophy of the retinal pigment epithelium) Li-Fraumini correct answers early breast, sarcoma, brain, leukemia, adrenocortical, choroid plexus tumors (childhood), fam hx childhood onset tumors Cowden correct answers Folliular Thyroid, Breast, uterine, macrocephaly, Lhermitte-Duclose disease (LDD)= cerebellar dysplastic gangliocytoma Cowden correct answers Papillomatous papules, trichilemmomas, Noncancerous thyroid lesions, hamartomatous intestinal polyps, lipomas, fibromas, Autism, Oral papillomas, Multiple mucocutaneous lesions , Esophageal glycogenic acanthosis Familial Adenomatous Polyposis correct answers colon cancers in 20s & 30s, Colon adenomas, upper GI, desmoid, osteoma, thyroid, brain, hepatoblastoma, Attenuated FAP correct answers Colon ca in 50s, NO CHRPE, maybe upper GI lesions MYH Associated Polyposis (MAP) correct answers sebaceous gland tumors, look like lynch MYH Associated Polyposis (MAP) correct answers RECESSIVE, at least 15 fewer than 100s, onset ~50s, adenomatous, some hyperplastic, 17% duodenal polyposis, 4% duodenal cancer, higher ovarian, bladder, skin Lynch syndrome/ Hereditary Non-Polyposis Colorectal Cancer (HNPCC) correct answers CRC ~45yo, right sided tumors, Extracolonic cancers: endometrial, stomach, ovarian, pancreas, ureter, renal pelvis (transitional cell ca), biliary tract, brain (usually glioblastoma as seen in Turcot syndrome), sebaceous gland adenomas, keratoacanthomas in Muir-Torre syndrome, carcinoma of the small bowel Constitutional Mismatch Repair correct answers RECESSIVE, very early CRC teens-20s, duodenal, leukemia, lymphoma, childhood brain, Café-au-lait spots, death by 20yo Familial Colorectal Cancer Type X correct answers Meet Amsterdam criteria, MSI normal, no other HNPCC tumors, later ages of onset, less 2nd tumors, unknown genes Muir-Torre correct answers HNPCC +, sebaceous gland adenomas, keratoacanthomas Turcot correct answers Colonic Polyposis, brain, usually glioblastoma, (in FAP= meduloblastomas)