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Pathology - Pheochromocytoma
Approximately 90% of instances occur irregularly. Other instances are linked to MEN IIA, MEN IIB, neurofibromatosis, or von Hippel-Lindau disease.
Pathology
Gross findings include variable alterations in the adrenal medulla, ranging from small confined lesions to massive hemorrhagic lesions with a lobular pattern.
The tumor is made up of clusters of polygonal chromaffin cells with catecholamine-rich granules. Occasionally, enormous, pleomorphic cells can be observed.
Symptoms and signs
Intermittent bouts of hypertension, headache, palpitations, and diaphoresis are caused by the release of epinephrine and norepinephrine from tumors.
Laboratory results: Elevated 24-hour urinary catecholamine and metanephrine levels, as well as elevated plasma metanephrine levels.
Therapies
Begin treatment with alpha-adrenergic blocking medications (phenoxybenzamine) and then proceed with surgical removal of the mass.
Pheochromocytomas exhibit the following characteristics: 10% are malignant, 10% are bilateral, 10% are familial, 10% are extra-adrenal, and 10% occur in youngsters. Pheochromocytomas can develop in locations other than the adrenal gland and are referred to as paragangliomas.
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