Pheochromocytoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]

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.

Gross Pathology

multinodular, multicentric pattern of growth of pheochromocytoma may be seen. Image:Bilateral pheo MEN2.jpg|Bilateral pheochromocytoma in MEN2. Gross image.

Microscopic Pathology

Pheochromocytoma typically demonstrate a nesting (Zellballen) pattern on microscopy. This pattern is composed of well-defined clusters of tumour cells containing eosinophilic cytoplasm separated by fibrovascular stroma.