Pheochromocytoma: Unmasking the chameleon

Abstract

Pheochromocytomas and extraadrenal paragangliomas are functional tumors of the neuroendocrine system. They can secrete excessive amounts of catecholamines and although signs and symptoms may vary, generally they present as hypertension accompanied by sympathetic signs and symptoms. Plasma or urine metanephrines are reliable biochemical tests that are sensitive to detect patients with biochemically active tumors. CT-scan and MRI are anatomic imaging tests that remain vital for accurate pre-operative assessment. Functional imaging can be diagnostic and therapeutic; it enables detection of tumor extent and assesses possible response to therapy. Focus on genetic studies for familial predisposition for early detection is now at the forefront of research. Identi fi cation of SDHB mutations warrants aggressive treatment because of the propensity of these tumors for metastases with poor prognosis. Surgery remains the cornerstone of treatment with potential cure for benign tumors and palliative management. Long-term follow-up is necessary to detect recurrences and metastases. Malignancy still holds poor prognosis as therapeutic options remain limited. Radiopharmaceutical and chemotherapy remains promising but no major breakthroughs have been achieved. Palliative care and lesion-based treatment remains the standard.