Pheochromocytoma

Pheochromocytoma (PHEO) is a tumor of chromaffin cells; extra-adrenal PHEO can occur anywhere from carotid body to pelvis. Causes hypertension and metabolic disturbances; severe HTN can damage heart/brain/kidney or cause crisis, but is curable if diagnosed early.

Common Symptoms

Shock; functional tremor; arrhythmia; nausea; weight loss; cardiomegaly; palpitations with hypertension; excess catecholamine secretion

Causes

80-90% are adrenal (mostly unilateral); extra-adrenal 10-15% (paraaortic), rare renal/hepatic hilum/bladder/rectal; mostly benign, 10% malignant. Sporadic cause unknown; familial linked to MEN-2A/2B (RET proto-oncogene chr10), VHL syndrome (3p25-26), neurofibromatosis type 1 (chr17). Path: adrenal PHEO usually <10 cm (3-5 cm), 10 g; round/oval, gray-brown cut with hemorrhage/necrosis/cyst/calcification; large polygonal chromaffin cells with epinephrine/norepinephrine granules. Extra-adrenal 15-20%, usually <5 cm, 20-40 g, along sympathetic chain/Zuckerkandl organ. Biochemical: most secrete NE; adrenal ones secrete E; VMA/HVA elevated. Familial: MEN-2A (40-50%), VHL (10-20%), NF1.

Treatment

Drug therapy; surgery; rehabilitation

Prevention

Suspect PHEO in: paroxysmal/sustained HTN with headache/palpitations/sweating/pallor/pain; accelerated/malignant HTN in children; unexplained shock/alternating BP; BP spikes during surgery/anesthesia/pregnancy/delivery; poor response to standard antihypertensives. Surgical resection is curative; maintain perioperative BP stability. Familial genetic workup.

Recommended Department

It is recommended to visit the endocrinology department; before the visit you may first book an appointment and connect with experts through this platform.

Warm reminder: The above information is provided for health education reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek care at a regular hospital in time.