Adrenal Tumors
Pheochromocytoma
Pheochromocytoma is a rare adrenal tumor causing excess catecholamine production, leading to severe hypertension and systemic effects.
Pheochromocytoma: Understanding Adrenal Tumors
Pheochromocytoma is a rare neuroendocrine tumor that originates from the adrenal medulla. It results in excessive production of catecholamines, leading to hypertension and other systemic effects.
Etiology and Pathogenesis
Pheochromocytomas arise from chromaffin cells within the adrenal gland. The tumor is associated with genetic syndromes such as Multiple Endocrine Neoplasia (MEN) type 2, Von Hippel-Lindau disease, and Neurofibromatosis type 1.
Clinical Presentation
- Severe hypertension
- Paroxysmal episodes of headache, sweating, and tachycardia
- Orthostatic hypotension
- Hyperglycemia
Diagnosis
Diagnostic workup includes measurement of plasma free metanephrines and urinary catecholamines. Imaging modalities such as CT and MRI help localize the tumor.
Management
The definitive treatment for pheochromocytoma is surgical resection. Preoperative alpha-blockade (e.g., phenoxybenzamine) is necessary to prevent hypertensive crises during surgery.
Prognosis
The prognosis is generally favorable following complete tumor removal. However, long-term follow-up is essential due to recurrence risk.
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