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Diagnostic Ultrasound - Abdomen and Pelvis

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Pheochromocytoma<br />

Diagnoses: Adrenal Gl<strong>and</strong><br />

TERMINOLOGY<br />

• Definition: Rare catecholamine-secreting tumor arising<br />

from chromaffin cells of adrenal medulla<br />

• Termed paraganglioma if extraadrenal<br />

IMAGING<br />

• Best diagnostic clue<br />

○ Adrenal mass in setting of clinical symptoms or<br />

biochemical abnormality<br />

– Paroxysmal headache, palpitations, sweating<br />

– ↑ levels of 24-hr urine-fractionated metanephrines<br />

• "Imaging chameleon": Variable US/CT/MR appearance;<br />

mimics other lesions<br />

○ Commonly solid <strong>and</strong> hypervascular ± cystic change,<br />

necrosis, <strong>and</strong> calcification<br />

○ Can be purely cystic<br />

• First-line: CT or MR<br />

• US: Comparable to CT in detecting adrenal tumors; limited<br />

for extraadrenal disease<br />

KEY FACTS<br />

• I-123 MIBG: For extraadrenal, metastatic, or recurrent<br />

disease<br />

TOP DIFFERENTIAL DIAGNOSES<br />

• Adrenal adenoma<br />

• Adrenal metastases or lymphoma<br />

• Adrenocortical carcinoma<br />

• Adrenal neuroblastoma<br />

• Adrenal granulomatous infection<br />

DIAGNOSTIC CHECKLIST<br />

• Remembered as "rule of 10s"<br />

○ 10% extraadrenal (paraganglioma)<br />

○ 10% bilateral (suggesting hereditary disease)<br />

○ 10% pediatric (also suggests hereditary disease)<br />

○ 10% contain calcification<br />

○ 10% malignant (higher for extraadrenal cases)<br />

○ 25% familial (previously thought to be 10%)<br />

(Left) Graphic shows a typical<br />

pheochromocytoma ,<br />

moderate in size, with a wellcircumscribed<br />

margin <strong>and</strong> solid<br />

appearance. Note<br />

hypervascularity ſt of the<br />

mass, which commonly results<br />

in necrosis <strong>and</strong> cystic change.<br />

(Right) Transverse<br />

transabdominal ultrasound<br />

demonstrates a wellcircumscribed,<br />

homogeneous<br />

hypoechoic adrenal mass ſt.<br />

The appearance is nonspecific,<br />

but the patient presented with<br />

elevated catecholamines <strong>and</strong><br />

hypertensive urgency,<br />

suggesting a diagnosis of<br />

pheochromocytoma.<br />

(Left) Longitudinal<br />

transabdominal ultrasound<br />

shows a well-demarcated,<br />

solid right adrenal mass ,<br />

which was proven to be a<br />

pheochromocytoma. It is<br />

hyperechoic to the renal<br />

cortex . (Right) Transverse<br />

transabdominal ultrasound of<br />

a right adrenal<br />

pheochromocytoma shows a<br />

large, well-defined echogenic<br />

mass with an anechoic<br />

cystic area representing<br />

necrosis .<br />

598

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