Highlights & Basics
- Pheochromocytoma is a rare neuroendocrine tumor of the adrenal glands. It can present with palpitations, diaphoresis, pallor, and paroxysmal hypertension.
- Risk factors include multiple endocrine neoplasia type 2, Von Hippel-Lindau syndrome, and neurofibromatosis type 1.
- Diagnosed by increased levels of metanephrines and normetanephrines.
- Treatment includes medical therapy for hypertension and surgical excision of tumor (open or laparoscopic adrenalectomy). For unresectable tumors, alternative approaches include chemotherapy or radiopharmaceuticals.
- Complications include hypertensive crisis, myocardial infarction, and hypotension.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Abdominal CT scan with mass in the left adrenal gland, compatible with a pheochromocytoma
Metaiodobenzylguanidine (MIBG) scintigraphy identified hyperfixation in the left adrenal gland compatible with pheochromocytoma
Left laparoscopic adrenalectomy: (A) macroscopic examination, 6 cm tumor; (B) microscopic examination: neoplasm of the adrenal medulla with eosinophilic cytoplasm of large cells with positive fine granular chromogranin A; (C) round and oval nucleus and sustentacular cells S100+; (D) pheochromocytoma
Citations
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Kunz PL, Reidy-Lagunes D, Anthony LB, et al; North American Neuroendocrine Tumor Society. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.[Abstract][Full Text]
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