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Multiple Endocrine Neoplasias for NP

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Multiple Endocrine Neoplasias for the Nurse Practitioner Licensing Exam
  • Pathophysiology
    • Multiple Endocrine Neoplasias (MEN) are inherited syndromes that lead to tumors in multiple endocrine glands. They are classified into three types: MEN1, MEN2A, and MEN2B.
    • MEN1: Caused by mutations in the MEN1 gene, leading to tumor development primarily in the parathyroid, pancreas, and pituitary glands. Loss of the tumor suppressor gene menin leads to uncontrolled cellular growth.
    • MEN2A and MEN2B: Both result from mutations in the RET proto-oncogene, which encodes a receptor tyrosine kinase, leading to medullary thyroid carcinoma (MTC), pheochromocytoma, and other endocrine tumors.
Multiple Endocrine Neoplasias
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MEN1
  • Clinical Features:
    • Primary Hyperparathyroidism: The most common feature, presenting as hypercalcemia, kidney stones, and bone pain.
    • Pituitary Adenomas: Typically prolactinomas (causing galactorrhea) or GH-secreting tumors (causing acromegaly).
    • Pancreatic Neuroendocrine Tumors (NETs): Include gastrinomas (causing Zollinger-Ellison syndrome) and insulinomas (causing hypoglycemia).
  • Management:
    • Parathyroidectomy for hyperparathyroidism.
    • Dopamine agonists for prolactinomas, and proton-pump inhibitors for gastrinomas.
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MEN2A
  • Clinical Features:
    • Medullary Thyroid Carcinoma (MTC): Caused by calcitonin-secreting C-cells, leading to a neck mass, diarrhea, or flushing.
    • Pheochromocytoma: Causes episodic hypertension, palpitations, and sweating.
    • Primary Hyperparathyroidism: Less common than in MEN1 but may lead to hypercalcemia.
  • Management:
    • Prophylactic Thyroidectomy in childhood to prevent MTC.
    • Adrenalectomy for pheochromocytoma.
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MEN2B
  • Clinical Features:
    • Medullary Thyroid Carcinoma: More aggressive than in MEN2A, often presenting early.
    • Pheochromocytoma: Causes similar symptoms as in MEN2A.
    • Mucosal Neuromas: Painless growths on lips, tongue, and other mucous membranes.
    • Marfanoid Habitus: Long limbs and joint hypermobility.
  • Management:
    • Early Thyroidectomy and adrenalectomy for tumor management.
    • --
Key Points
  • MEN1 involves tumors in the parathyroid, pancreas, and pituitary glands. Hyperparathyroidism is the most common manifestation.
  • MEN2A and MEN2B are caused by RET mutations, associated with medullary thyroid carcinoma and pheochromocytoma. MEN2B is more aggressive and includes mucosal neuromas.
  • Early thyroidectomy is critical in MEN2 to prevent medullary thyroid carcinoma.

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