Endocrine
MEN Syndromes & Endocrine Tumors
Endocrine

MEN Syndromes & Endocrine Tumors

MEN 1, 2A, 2B + carcinoid + insulinoma + gastrinoma.

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◆Functional pancreatic NETs

  • •Insulinoma: hypoglycemia (Whipple triad) + ↑insulin + ↑C-peptide (vs exogenous → low C-peptide); 72-hr fast
  • •Gastrinoma (ZES): multiple recurrent ulcers + diarrhea + weight loss; ↑gastrin off PPI + secretin stim
  • •VIPoma: WDHA syndrome (Watery Diarrhea + Hypokalemia + Achlorhydria)
  • •Glucagonoma: necrolytic migratory erythema + diabetes + diarrhea + DVT
  • •Somatostatinoma: diabetes + steatorrhea + gallstones

◆Carcinoid syndrome

  • •Serotonin-secreting NET — usually metastatic to liver to bypass hepatic clearance
  • •Flushing + diarrhea + bronchospasm + right-sided valvular disease (TR, PS)
  • •↑ 24-hr urine 5-HIAA
  • •Localize: CT, octreotide scan
  • •Treat: octreotide + surgical resection

Multiple endocrine neoplasia syndromes (all AD)

SyndromeGeneTumors
MEN 1 (3 P's)MEN1 (menin)Pituitary adenoma + Parathyroid hyperplasia + Pancreatic NETs (gastrinoma, insulinoma, glucagonoma, VIPoma)
MEN 2A (2 P's + 1 M)RETMedullary thyroid CA + Pheochromocytoma + Parathyroid hyperplasia
MEN 2B (1 P + 2 M)RETMedullary thyroid CA + Pheochromocytoma + Mucosal neuromas + marfanoid habitus

High-yield pearls

  • ◆Medullary thyroid cancer → screen for pheochromocytoma BEFORE surgery (must α-block first)
  • ◆Prophylactic thyroidectomy for kids with RET mutation (especially MEN 2B)
  • ◆Whipple triad for insulinoma: hypoglycemia + symptoms + relief with sugar
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