Pheochromocytoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

Traditionally it is known as the "10% tumor":

  • bilateral disease is present in approximately 10% of patients
  • approximately 10% of tumours are malignant
  • approximately 10% are located in chromaffin tissue outside of the adrenal gland
  • Approximately 10% arise in childhood
  • Approximately 10% are familial
  • Approximately 10% recur after being resected
  • Approximately 10% patients do not have hypertension (Campbell's Urology)These tumors can form a pattern with other endocrine gland cancers which is labeled multiple endocrine neoplasia (MEN). Pheochromocytoma may occur in patients with MEN 2 and MEN 3. VHL (Von Hippel Lindau) patients may also develop these tumors.

Tumor Location

In adults, 90% tumors are located unilaterally and are solitary, and 10% are located outside the adrenal gland. In children 50% are adrenal, while 25% are bilateral and 25% are extraadrenal. The common extradrenal locations are the abdomen, thorax and urinary bladder.

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