pheokromosytooman

Pheokromosytooman, also known as pheochromocytoma, is a rare neuroendocrine tumor that originates from chromaffin cells in the adrenal medulla or extra-adrenal paraganglia. It secretes catecholamines (epinephrine, norepinephrine, dopamine), leading to episodic or sustained hypertension and other symptoms.

Most arise in the adrenal glands but about 10–15% are extra-adrenal (paragangliomas). Incidence is roughly 2–8 per million per year. Approximately 30–40% are familial; genes include RET (MEN2), VHL, NF1, SDHB, SDHD.

Clinical features commonly include a triad of headaches, sweating, and palpitations; patients may experience episodic hypertension, tachycardia, anxiety, and tremor. Hypertensive crises can be precipitated by stress, surgery, or anesthetic exposure. Some tumors secrete predominantly dopamine and may present with hypotension rather than hypertension.

Diagnosis relies on biochemical testing and imaging. Preferred biochemical tests measure plasma free metanephrines or 24-hour urine fractionated metanephrines. Localization is typically achieved with CT or MRI, with functional imaging (MIBG scintigraphy or PET scans) used in ambiguous cases or for suspected metastases.

Treatment centers on surgical resection. Preoperative management includes alpha-adrenergic blockade (such as phenoxybenzamine or doxazosin) to control blood pressure and volume status, followed by beta-blockade if needed after adequate alpha blockade. Surgery is curative for localized disease; malignant or metastatic cases may require systemic therapy, radiotherapy, or targeted approaches. Genetic counseling is recommended given the heritable associations.

Prognosis is favorable for localized tumors after complete resection, but recurrence and metastasis can occur. Lifelong follow-up with periodic biochemical testing and imaging is advised.