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 aged 20-50 years.
Rarely, Pacak-Zhuang syndrome may be seen in patients with early-onset polycythemia and paragangliomas.
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%.
Related topics: von Hippel-Lindau disease, multiple endocrine neoplasia type 2A, multiple endocrine neoplasia type 2B
C74.10 – Malignant neoplasm of medulla of unspecified adrenal gland
302835009 – Pheochromocytoma
Differential Diagnosis & Pitfalls
- Primary hypertension
- Use of medications or illicit drugs that may increase catecholamines and metanephrines (see sympathomimetic toxicity)
- Alcohol withdrawal
- Other adrenal masses such as adenoma, adrenal hemorrhage, myelolipoma, carcinoma, or metastases – Need to perform biochemical testing for pheochromocytoma before any surgery or procedure of adrenal mass.
- Other causes of "spells" such as menopausal symptoms, flushing, hypoglycemia, postural orthostatic tachycardia syndrome, carcinoid syndrome, or mast cell disease (eg, systemic mastocytosis)