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🌱 來自: adrenal disorders

pheochromocytoma

Clinical manifestations (NEJM 2019;381:552)

  • Neuroendocrine neoplasm leads to inappropriate and paroxysmal release of adrenergic agents including epinephrine, norepinephrine, and rarely dopamine

  • Classic triad: 頭痛、心悸、流汗

    • episodic headaches, palpitations and profuse sweating;
    • only 50% have paroxysmal hypertension and most Pts do not have three classic sx
  • Paroxysms can be triggered by meds (eg, β-blockers 會引發), abdominal manipulation

  • Up to 40% of pheos/paragangliomas thought to have underlying genetic etiology; genetic testing frequently recommended

  • Associated with

    • MEN2A/2B MEN2
    • von Hippel-Lindau
    • NF1
    • familial paraganglioma (mutations in succinate dehydrogenase gene B, C and D),
    • MAX or TMEM127 mutations
  • Diagnostic studies of pheochromocytoma 󰒗

  • Treatment

    • α-blockade first (usually phenoxybenzamine) ± β-blockade (often propranolol) → surgery
    • Preoperative volume expansion is → critical due to possible hypotension after tumor excision