**Core Concept**
Multiple endocrine neoplasia (MEN) syndromes are rare genetic disorders characterized by the occurrence of tumors in multiple endocrine glands. MEN 1 (Wermer syndrome) is a distinct entity that primarily affects the parathyroid glands, pancreatic islet cells, and pituitary gland.
**Why the Correct Answer is Right**
MEN 1 syndrome is caused by mutations in the MEN1 gene, leading to the development of hyperparathyroidism in approximately 80% of cases due to parathyroid gland hyperplasia. Pancreatic islet cell tumors are another common feature, often secreting hormones such as gastrin, insulin, or vasoactive intestinal polypeptide (VIP). Pituitary adenomas can also occur, secreting various hormones like prolactin or growth hormone. The combination of these endocrine tumors is characteristic of MEN 1 syndrome.
**Why Each Wrong Option is Incorrect**
**Option B:** MEN 2A (Sipple syndrome) is primarily associated with medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism, but not with pancreatic islet cell tumors or pituitary adenomas. MEN 2A is caused by mutations in the RET proto-oncogene.
**Option C:** MEN 2B is characterized by medullary thyroid carcinoma, pheochromocytoma, and mucosal neuromas, but not with the endocrine tumors seen in MEN 1 syndrome.
**Clinical Pearl / High-Yield Fact**
MEN 1 syndrome is an autosomal dominant disorder, meaning that a single mutated allele is sufficient to cause the disease. Patients with MEN 1 syndrome should undergo regular screening for hyperparathyroidism, pancreatic islet cell tumors, and pituitary adenomas to enable early diagnosis and treatment.
**β Correct Answer: A. Men 1 (Wermer)**
Free Medical MCQs Β· NEET PG Β· USMLE Β· AIIMS
Access thousands of free MCQs, ebooks and daily exams.
By signing in you agree to our Privacy Policy.