ABGC Boards - Final Review 2024 (100% Verified with correct answers)

Hereditary Breast and Ovarian Cancer correct answers BRCA 1/2 Breast 50-87% Second primary tumor 50-64% BRCA 1: 20-45% BRCA 2: 10-30% BRCA 2: Male breast 6-10% Li-Fraumeni Syndrome correct answers -Sarcoma, breast, leukemia, adrenal and many others -May present in almost every way (ie all family members with same cancers or all with different) -Adrenocortical carcinoma considered pathognomonic -50% have some type by 30, 90% by age 60 -TP53 accounts for 70% of individuals Beckwith-Wiedemann correct answers Overgrowth disorder in children, tongue, viscera, hemihyperplasia, abdominal wall defects, ear pits and creases. Pancreatic hyperplasia leads to neonatal hypoglycemia and MR Airway and feeding difficulties, Methylation abnormalities/imprinting disorder 11p15.5 (need mom, dad is shut off) -Childhood embryonal tumors (7.5%), Wilms (Kidney), hepatoblastoma, neuroblastoma, rhabdomyosarcoma hepatoblastoma correct answers malignant liver tumor, most common primary liver malignancy in children, associated with BWS Wilms tumor correct answers AKA kidney tumor, associated with BWS oncogene correct answers A gene having the potential to cause a normal cell to become cancerous. Lynch Syndrome correct answers cancer predisposition syhndrome affecting increased 50-80% risk for colon cancer before age 50 caused by germline pathogenic variant in one of four mismatch repair genes (MMR) -MSH2 -MSH6 -MLH1 -PMS2 MLH1 and MSH2 variants account for approximately 90% of pathogenic variants