Presentation and outcomes of adrenal ganglioneuromas: A cohort study and a systematic review of literature.

Objective To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGN). Design Single-center retrospective cohort study (January 1st , 1995 to December 31st , 2019) and systematic review of literature (January 1st , 1980 to November 19th , 2019). Patients Diagnosed with histologically confirmed AGN. Measurements Baseline clinical, imaging, and biochemical characteristics, recurrence rates, and mortality. Sub-group analysis was performed on tumors with histologic elements of ganglioneuroma and pheochromocytoma (i.e. composite tumors). Results The cohort study included 45 patients with AGN, 20 (44%) of which had composite tumors. Compared to pure AGN, patients with composite tumor were older (median age, 62.5 vs. 35 years, P Conclusions AGNs are rare benign tumors that present with variable imaging features including large size, unenhanced CT attenuation >20 HU, calcifications, and lobulated shape. Imaging characteristics can assist in establishing a diagnosis and avoiding an unnecessary adrenalectomy. The association of pheochromocytomas with AGNs is frequent. Diagnosis should include biochemical testing.