A 25-year-old man develops paroxysmal hypertension, diaphoresis, and palpitations. 24-hour urine shows markedly elevated catecholamines and metanephrines. Imaging reveals a 4 cm adrenal mass. This tumor arises from which cell type?
- A Adrenocortical cells of the zona fasciculata
- B Sustentacular (Schwann) cells of the adrenal medulla
- C Juxtaglomerular cells of the kidney
- D Chromaffin cells of the adrenal medulla (neural crest-derived) ✓
Explanation
Pheochromocytoma arises from chromaffin cells of the adrenal medulla (and extra-adrenal paragangliomas from paraganglia), which are neural crest-derived cells that synthesize and secrete catecholamines (epinephrine, norepinephrine, dopamine). The 'rule of 10s' applies: ~10% bilateral, 10% extra-adrenal, 10% malignant, 10% familial. Sustentacular cells are supportive stromal cells within the tumor (S100+). Cortical tumors cause hyperaldosteronism or Cushing syndrome; juxtaglomerular cells produce renin.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
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