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Pheochromocytoma
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Pheochromocytoma

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Contributors: Abhijeet Waghray MD, Paritosh Prasad MD
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Synopsis

Pheochromocytoma (PCC) is a rare catecholamine-secreting neoplasm of adrenal or extra-adrenal origin. The most common presenting symptoms are hypertension (including labile hypertension), palpitations, increased sweating, severe headache, tremors, pallor, and dyspnea. Atypical symptoms include anxiety, abdominal pain, constipation, insomnia, and weight loss. The prevalence of PCC in patients with hypertension is less than 0.2%. Clinical presentation is characterized by transient duration of symptoms (eg, 15-20 minutes) that may recur several times a day. Physical exertion (moderate to extreme exercise) or bowel movements may provoke symptoms.

The majority of PCCs are intra-adrenal and solitary. Approximately 10% of all catecholamine-secreting tumors will be malignant. PCC may occur at any age but has the highest prevalence in individuals 20-50 years of age.

Treatment involves surgical removal of the tumor after adequate medical therapy is provided, generally with combined alpha- and beta-adrenergic blockade. Tumors can recur in less than 10% of patients. Surgery may help return norepinephrine and epinephrine levels to normal. Despite perioperative management, acute hypertensive crises may develop during surgical resection. Management of acute hypertensive crisis due to pheochromocytoma includes nitroprusside, phentolamine, or nicardipine.

In patients with malignant tumors who have undergone surgery, the 5-year survival rate is less than 50%.

For more information, see OMIM.

Related topics: von Hippel-Lindau disease, Multiple endocrine neoplasia type 2A, Multiple endocrine neoplasia type 2B

Codes

ICD10CM:
C74.10 – Malignant neoplasm of medulla of unspecified adrenal gland

SNOMEDCT:
302835009 – Pheochromocytoma

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Last Reviewed: 03/07/2018
Last Updated: 04/19/2018
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Pheochromocytoma
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Pheochromocytoma : Headache, HR increased, BP increased, Excessive sweating
Imaging Studies image of Pheochromocytoma
Patient presented with labile blood pressure. Unenhanced CT scan of the abdomen demonstrates a large complex right adrenal mass, surgically proven pheochromocytoma.
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