Which syndrome is associated with the presence of pancreatic, pituitary, and parathyroid tumors?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

The correct answer pertains to Multiple Endocrine Neoplasia type 1 (MEN 1), also known as Wermer’s syndrome. This syndrome is characterized by a triad of tumors involving the pancreas (often insulinomas or gastrinomas), the pituitary gland (often secreting growth hormone or prolactin), and the parathyroid glands (leading to primary hyperparathyroidism due to parathyroid adenomas).

MEN 1 is a genetic disorder that follows an autosomal dominant inheritance pattern, caused by mutations in the MEN1 gene, which encodes the protein menin. This protein is involved in various cellular functions, including cell division and gene expression, thus leading to the development of tumors when it is dysfunctional.

The association of these three gland tumors is a hallmark of MEN 1. Other syndromes mentioned do not share this specific combination of tumors. For example, MEN 2A primarily features medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism, while MEN 2B presents medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, and a marfanoid habitus. Li-Fraumeni syndrome is characterized by a predisposition to a variety

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