Adrenocortical Carcinoma Associated With Multiple Endocrine Neoplasia Type 1

Although adrenocortical nodularity is fairly common in patients with multiple endocrine neoplasia type 1 (MEN-1), adrenocortical carcinoma (ACC) is rare. The case presented herein is a cautionary notice for clinicians who have patients with small lipid-poor adrenal masses that are thought to be benign adrenal adenomas.

Case Report

This 51-year-old man came to Mayo Clinic for treatment of recurrent left adrenal ACC. He had been diagnosed with MEN-1 21 years previously when he presented with a duodenal gastrinoma and primary hyperparathyroidism—both of which were treated effectively surgically. Subsequently, he was diagnosed with a pituitary prolactinoma, facial angiofibromas, left hip lipoma, and left adrenal “adenoma.” His mother and maternal grandmother had previously been diagnosed with MEN-1. Germline genetic testing was not performed.

The left adrenal tumor was first detected on a computed tomography (CT) scan 11 years previously ( Fig. 32.1 ). At that time, it was 1.6 cm in diameter with an unenhanced CT attenuation of 39.1 Hounsfield units (HU). Enhanced CT imaging completed at 4 and 5 years after the initial discovery showed the left adrenal mass to be slightly larger at 1.8 cm (see Fig. 32.1 ). Laboratory evaluation showed normal levels of plasma fractionated metanephrines, serum cortisol, 24-hour urinary free cortisol, and dehydroepiandrosterone sulfate (DHEA-S). Two years before coming to Mayo Clinic he underwent left adrenalectomy after the left adrenal mass had markedly enlarged. However, details of the operation, preoperative imaging, and pathologic examination were not available for our review.

INVESTIGATIONS

The image labeled “11 yrs” in Fig. 32.1 was obtained after his adrenal surgery and 3 months before his Mayo Clinic consultation. Magnetic resonance imaging (MRI) at Mayo Clinic showed numerous nodular deposits of locally recurrent tumor in the splenectomy and distal pancreatectomy bed with extension into the renal hilum and renal cortex and more than five bilobar hepatic metastases; the largest lesion in hepatic segment VIII measured 2.7 cm in maximal diameter ( Fig. 32.2 ). All adrenocortical function testing was normal.