Pheochromocytoma

Pheochromocytoma is a rare, typically benign neuroendocrine tumor arising from chromaffin cells in the adrenal medulla or extraadrenal sympathetic ganglia, characterized by excessive secretion of catecholamines—primarily epinephrine—leading to episodic or sustained hypertension, tachycardia, severe headaches, profuse sweating, palpitations, and anxiety. These tumors are vascular in nature and can be associated with hereditary syndromes such as MEN2, NF1, and other neurocristopathies, following Knudson’s two-hit hypothesis for tumorigenesis. While most pheochromocytomas are non-malignant, they may occasionally exhibit malignant behavior. Diagnosis relies on clinical presentation, biochemical testing for elevated catecholamines and metabolites, and imaging studies. Treatment primarily involves surgical resection, with adjuvant therapies including radiation, chemotherapy, and targeted agents considered in advanced or metastatic cases.