MEN1, MEN2A and MEN2B genes and components, carcinoid syndrome, insulinoma, gastrinoma and other pancreatic neuroendocrine tumors, and thyroid cancer types.
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- Which gene is mutated in MEN1 syndrome?
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The MEN1 gene
- What is the inheritance pattern of MEN1 syndrome?
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Autosomal dominant
- What protein does the MEN1 gene encode?
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Menin
- What type of protein is menin?
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A tumor suppressor
- What is the most common manifestation of MEN1 syndrome?
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Primary hyperparathyroidism, from parathyroid hyperplasia
- What is the most common type of pituitary tumor seen in MEN1?
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Prolactinoma
- What is the most common functional pancreatic neuroendocrine tumor in MEN1?
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Gastrinoma
- What is the second most common pancreatic neuroendocrine tumor in MEN1?
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Insulinoma
- MEN1 syndrome classically affects the ____, remembered as the three P's.
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parathyroid, pituitary, and pancreas
- Which gene is mutated in MEN2A and MEN2B syndromes?
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The RET proto-oncogene
- What type of mutation in RET causes MEN2 syndromes?
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A gain-of-function mutation
- What three components make up MEN2A syndrome?
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Medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism
- What tumor occurs in nearly all patients with MEN2A?
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Medullary thyroid carcinoma
- What components make up MEN2B syndrome?
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Medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, and marfanoid habitus
- What distinguishes MEN2B from MEN2A?
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MEN2B has mucosal neuromas and marfanoid habitus instead of hyperparathyroidism