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Pheochromocytoma

Archie R McGowan, MD
Douglass F Adams, MD

1995

Presentation

A 41-year-old woman presented to her endocrinologist with a history of intermittent headaches and nosebleeds. She had previously undergone thyroidectomy for carcinoma. Examination during a headache revealed tachycardia and hypertension.

Imaging Findings

Contrast-enhanced axial CT
T1-weighted axial MRI
T2-weighted axial MRI

A contrast-enhanced axial computed tomogram (CT) at the level of the left adrenal shows a mass (not of fat attenuation) (black arrow) replacing the left adrenal gland. Metallic clips (white arrows) are visible in the contralateral adrenal bed.

An axial T1-weighted magnetic resonance image (MRI) at the level of the left adrenal demonstrates the same abnormality. A well circumscribed spherical mass of homogeneous intermediate signal intensity is visible in the left adrenal bed (arrow).

An axial T2-weighted image at the same level reveals the mass to have a high T2 signal intensity (light bulb-like) (arrow).

Differential Diagnosis

Differential diagnosis of adrenal masses: Rare causes:

Diagnosis

Pheochromocytoma

Discussion

This individual had a history of medullary cell carcinoma of the thyroid, and ten years earlier she had had a resection of her contralateral adrenal gland for a pheochromocytoma (hence the clips in the opposite adrenal bed). Interestingly, her familial history includes "cancer of the adrenals."

The presentation of medullary thyroid carcinoma with pheochromocytoma is the clinical syndrome of Multiple Endocrine Neoplasia (MEN) Type II. MEN IIA includes medullary cell carcinoma of the thyroid, pheochromocytoma, and parathyroid hyperplasia. MEN IIB includes orofacial neuroma, medullary cell carcinoma of the thyroid, and pheochromocytoma. Both syndromes follow autosomal dominant inheritance with molecular genetic abnormalities. Note that 7-10% of MEN presentations occur without family history and likely represent new mutations.

Hypertension is the most commonly encountered presenting symptom of pheochromocytoma, being at least episodically present in 91% of patients. Less than 1% of hypertension is the result of pheochromocytoma, however. Hypertensive paroxysms have been described as provoked by anesthesia, systemic intravenous iodinated contrast, selective angiography and surgical interventions.

Pheochromocytoma is said to follow the "rule of tens" with 10% found extra-adrenal, 10% being malignant neoplasms, and 5%-10% occurring bilaterally.

The classic MR image of pheochromocytoma is an adrenal mass that is intermediate on T1- and bright on T2-weighted images (light bulb), just as this patient's scan demonstrates. Some pheochromocytomas are, however, not bright on T2-weighted images or may be heterogeneously bright.

References

1. Bartley PL, Lloyd HM, Aitkens RE. Medullary carcinoma of the thyroid, multiple pheochromocytomas (Sipple's syndrome). Medical Journal of Australia 1976 July 31; 2(5):173-6.

2. Putnam C, Raven C. Textbook of diagnostic imaging. Philadelphia: Saunders Co, 1988; 1146-1150.

3. Rosenberg. Cancer principles and procedures in oncology. 3rd ed. Philadelphia: JB Lippincott Co; 1286-1303.

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