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Adrenal Pheochromocytoma
Etiology

Mutation in the RET oncogene (a receptor tyrosine kinase) or the MEN2 gene.
Pathogenesis

Unknown
Overproduction of unregulated catechols causes striking clinical symptoms,
Epidemiology

800 cases/year in the U.S.
Sporadic accounting for <0.3% of hypertension or
Associated with von Hippel-Lindau syndrome or
Associated with multiple endocrine neoplasia (MEN) type IIa or
May be associated with neurofibromatosis I
General Gross Description

Located in the adrenal medulla surrounded by rim of normal compressed adrenal
Vary in size
Usually hemorrhagic and necrotic
Dichromate fixatives oxidize catechols to brown black pigment
•Examples:
Pheochromocytoma (Cut Surface) Pheochromocytoma (Cut Surfaces)
General Microscopic Description

Cells with frequently pleomorphic nuclei and brown secretory vacuoles
Richly vascular stroma
Cells in cords or nests
Cytologic atypia irrelevant to diagnosis of malignancy
•Examples:
References

Cotran RS, Kumar V, Robbins SL. Robbins Pathologic Basis of Disease. 5th edition. Philadelphia, W.B. Saunders, 1994, pp. 1162-3

For Most Current Information Search Medline at National Library of Medicine
This link will directly take you to the relevant new literature
Adrenal Pheochromocytoma
Synopsis by: Melinda Sanders M.D. (T93000M87000)[152]
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