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The most likely diagnosis in this patient is Zollinger-Ellison syndrome (ZES) in the setting of underlying multiple endocrine neoplasia type 1 (MEN 1). MEN 1 occurs due to a mutation in the MEN1 gene on chromosome 11. EMEN 1 is an autosomal dominant cancer syndrome that predisposes individuals to neoplasms in the pituitary gland, parathyroid gland, and endocrine pancreas. Specifically, MEN1 increases the risk of pancreatic islet cell/endocrine tumors (e.g., ZES, insulinoma, glucagonoma, and VIPoma), parathyroid adenomas, and hormone-secreting pituitary tumors (e.g., prolactinomas and acromegaly). Individuals with MEN 1 have a mutation in the MEN1 gene which encodes for the tumor suppressor protein, menin. The MEN1 gene is found on chromosome 11. Incorrect Answers: Answer 1: Chromosome 5 contains the tumor suppressor gene, APC. Mutations in APC are associated with familial adenomatous polyposis (FAP), a cancer syndrome predisposing individuals to colon polyps and colorectal cancer. Answer 2: Chromosome 10 contains the RET proto-oncogene. Mutations in RET are associated with MEN 2A and 2B. MEN 2A is characterized by medullary thyroid carcinoma, pheochromocytoma, and parathyroid tumors. MEN 2B is characterized by medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, and a Marfanoid habitus. Answer 4: Chromosome 13 contains the tumor suppressors RB and BRCA2. Mutations in RB are associated with retinoblastoma and osteosarcoma. Mutations in BRCA2 are associated with an increased risk of breast and ovarian cancers. Answer 5: Chromosome 17 contains the tumor suppressors p53, BRCA1, and NF1. Mutations in p53 are associated with an increased risk of several tumors throughout the body. Mutations in BRCA1 are associated with an increased risk of breast and ovarian cancers. A mutation in NF1 is associated with neurofibromatosis type 1, which is characterized by the presence of café-au-lait spots and neurofibromas. Bullet Summary: Multiple endocrine neoplasia type 1 occurs due to an autosomal dominant mutation in the MEN1 gene on chromosome 11.
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