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

Pheochromocytoma arise from chromaffin cells of the adrenal medulla and sympathetic ganglia. Mutations of the genes VHL, RET, NF1, SDHB, and SDHD are all known to cause familial pheochromocytoma. The most common extradrenal locations are the abdomen and thorax .

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

Malignant and benign pheochromocytomas are the same; The only difference is the ability to spread locally and distant. ^[1]

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.

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.

Micrograph of pheochromocytoma.
Histopathology of adrenal pheochromocytoma. Adrenectomy specimen.
Micrograph of pheochromocytoma.
Micrograph of pheochromocytoma.

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References

↑ Goldstein RE, O'Neill JA, Holcomb GW, Morgan WM, Neblett WW, Oates JA; et al. (1999). "Clinical experience over 48 years with pheochromocytoma". Ann Surg. 229 (6): 755–64, discussion 764-6. PMC 1420821. PMID 10363888.

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[pmid10363888-1] Goldstein RE, O'Neill JA, Holcomb GW, Morgan WM, Neblett WW, Oates JA; et al. (1999). "Clinical experience over 48 years with pheochromocytoma". Ann Surg. 229 (6): 755–64, discussion 764-6. PMC 1420821. PMID 10363888.

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