Genitourinary Radiology > Adrenal Glands > Pheochromocytoma


Pheochromocytoma


Pheochromocytoma is a neoplasm of chromaffin cells that store and release excess amounts of catecholamines, which, in its extreme form, can cause sudden death due to hypertensive crisis, shock, or both. 5% are inherited, associated with multiple endocrine neoplasia (MEN) IIA and IIB, von Hippel-Lindau, and type I neurofibromatosis. When associated with MEN, they tend to be multicentric and frequently bilateral. 10% of pheochromocytomas are bilateral, 10% are extraadrenal, and 10% are malignant. Clinically, they present with elevated blood pressure, palpitations, perspirations, and headache.

On CT imaging, they are usually greater than 2 cm at diagnosis, with areas of central necrosis in larger tumors. These tumors may simulate, and therefore need to be differentiated from, a cystic adrenal mass. They exhibit homogenous attenuation with avid enhancement on CT or MRI.

Contrast enhanced CT shows an enhancing left adrenal mass (A) in this 9 year old patient with von Hippl Lindau.  Given the patients history, this lesion was resected (not his right adrenal gland had been resected earlier for pheochromocytoma).  The lesion is enhancing relatively intensely, a common finding with pheochromocytoma.

MRI of 37 yo male with von Hippl Lindau disease and elevated urinary catecholamines shows left adrenal mass with characteristic findings of pheochromocytoma. The left adrenal mass (arrows) is intermediate signal on T1 weighted images, is very high signal on T2 weighted images and enhances intensely on post gadolinium contrasted images.

The left adrenal mass (arrow) is intermediate signal on T1 weighted coronal image (1) and shows intense enhancement on post gadolinium contrasted images (2).



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