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

On gross pathology, pheochromocytoma has a multinodular and a multicentric pattern of growth. On microscopic histopathological analysis, nesting (Zellballen) pattern composed of well-defined clusters of tumor cells separated by fibrovascular stroma is a characteristic finding.

Pathophysiology

Mutations of the genes VHL, RET, NF1, SDHB, and SDHD are all known to cause familial pheochromocytoma. 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 most common extradrenal locations are the abdomen, thorax and urinary bladder.

Traditionally pheochromocytoma known as the "10% tumor":

• Approximately 10% of patients have bilateral disease
• Approximately 10% of tumors are malignant
• Approximately 10% are located in chromaffin tissue outside of the adrenal gland
• Approximately 10% occur in childhood
• Approximately 10% are familial
• Approximately 10% recur after being resected
• Approximately 10% of patients do not have hypertension

Pheochromocytoma can occur in patients with multiple endocrine neoplasia (MEN 2 and MEN 3). Patients with Von Hippel Lindau (VHL) may also develop pheocromocytoma.

Gross Pathology

A multinodular and multicentric pattern of growth of pheochromocytoma may be seen.

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  Bilateral pheochromocytoma in MEN2. Gross image.

Microscopic Pathology

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

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  Micrograph of pheochromocytoma.

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  Histopathology of adrenal pheochromocytoma. Adrenectomy specimen.

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  Micrograph of pheochromocytoma.

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  Micrograph of pheochromocytoma.

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