Incidental Bilateral Adrenal Ganglioneuromas

Presentation Number: SAT 381
Date of Presentation: April 1st, 2017

Britany Faith Epstein*1, Hans Kumar Ghayee2, Diana Barb1, Christiana Shaw3 and Robert W. Allan4
1University of Florida College of Medicine, Gainesville, FL, 2Malcom Randall VAMC, Gainesville, FL, 3uUniversity of Florida College of Medicine, 4University of Florida College of Medicine

Abstract

Background: Ganglioneuromas are tumors that arise from the paravertebral sympathetic ganglia. Adrenal ganglioneuromas (AGN) are rare and comprise 0-6% of all adrenal incidentalomas and 20% of all ganglioneuromas.

Clinical Case: 51-year-old male presented to the emergency room after a motor vehicle accident with left lower quadrant abdominal pain. On computed tomography (CT) scan of abdomen and pelvis it was noted that he had a contusion of the sigmoid colon as well bilateral lobular heterogeneously enhancing right greater than left adrenal masses, with patchy and rim-like areas of calcifications in both adrenals. The size of the right measured 9.5 x 5.1

x 6.1 cm and left measured 3.2 x 2.8 x 3.2 cm.

Biochemical work up revealed normal cortisol dynamics, no evidence of hyperaldosteronism or pheochromocytoma. However he was found to have slight elevation in urine dopamine levels that were less than two times the upper limit of normal.

CT guided biopsy of the right adrenal mass showed mature neural stroma with mixed ganglion cells consistent with a ganglioneuroma. After discussion with the patient and in tumor board, conservative management was chosen. A follow up CT scan three months later showed stability of the adrenal masses.

Although rare, adrenal ganglioneuromas can be found incidentally on imaging studies. Diagnosis is proven by tissue pathology, as there is a vast differential diagnosis once an adrenal mass is found on radiographic imaging. AGN are often hormonally silent or may sometimes secrete hormones. Such hormones include testosterone or if it is a composite tumor with pheochromocytoma, it may secrete catecholamines. In such cases, the diagnosis is challenging.

Conclusion: AGN needs to be considered in the differential diagnosis of an adrenal incidentaloma. Careful history, physical exam, biochemical testing, along with pathological examination is fundamental in making a diagnosis. AGN are benign and usually asymptomatic but need to be monitored for changes in size.

 

Nothing to Disclose: BFE, HKG, DB, CS, RWA