Kelley N Dages1, Jacob D Kohlenberg1,2, William F Young1,2, Mohammad Hassan Murad3, Larry Prokop4, Michael Rivera5, Benzon Dy6, Trenton Foster6, Melanie Lyden6, Travis McKenzie6, Geoffrey Thompson6, Irina Bancos1,2. 1. Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA. 2. Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA. 3. Evidence-Based Practice Center, Center for the Science of Healthcare Delivery, Mayo Clinic, Rochester, MN, USA. 4. Mayo Clinic Libraries, Mayo Clinic, Rochester, MN, USA. 5. Department of Laboratory Medicine and Pathology, Anatomic Pathology, Mayo Clinic, Rochester, MN, USA. 6. Division of Breast, Endocrine, Metabolic and GI Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, USA.
Abstract
OBJECTIVE: To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs). DESIGN: Single-centre retrospective cohort study (1 January 1995 to 31 December 2019) and systematic review of literature (1 January 1980 to 19 November 2019). PATIENTS: Diagnosed with histologically confirmed AGN. MEASUREMENTS: Baseline clinical, imaging and biochemical characteristics, recurrence rates and mortality. Subgroup analysis was performed on tumours with histologic elements of ganglioneuroma and pheochromocytoma (ie composite tumours). RESULTS: The cohort study included 45 patients with AGN, 20 (44%) of which had composite tumours. Compared to pure AGN, patients with composite tumour were older (median age, 62.5 vs. 35 years, p < .001), had smaller tumours (median size, 3.9 vs. 5.7 cm, p = .016) and were discovered incidentally less frequently (65% vs. 84%, p = .009). No recurrences or ganglioneuroma-specific mortality occurred during follow-up (range, 0-266 months). The systematic review included 14 additional studies and 421 patients. The mean age of diagnosis was 39 years, and 47% were women. AGNs were discovered incidentally in 72% of patients, were predominantly unilateral (99%) and had a mean diameter of 5.8 cm and an unenhanced computed tomography (CT) attenuation of -118 to 49 Hounsfield units (HU). On imaging, 69% of AGNs were homogenous, 41% demonstrated calcifications, and 40% were lobulated. CONCLUSIONS: AGNs are rare benign tumours 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.
OBJECTIVE: To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs). DESIGN: Single-centre retrospective cohort study (1 January 1995 to 31 December 2019) and systematic review of literature (1 January 1980 to 19 November 2019). PATIENTS: Diagnosed with histologically confirmed AGN. MEASUREMENTS: Baseline clinical, imaging and biochemical characteristics, recurrence rates and mortality. Subgroup analysis was performed on tumours with histologic elements of ganglioneuroma and pheochromocytoma (ie composite tumours). RESULTS: The cohort study included 45 patients with AGN, 20 (44%) of which had composite tumours. Compared to pure AGN, patients with composite tumour were older (median age, 62.5 vs. 35 years, p < .001), had smaller tumours (median size, 3.9 vs. 5.7 cm, p = .016) and were discovered incidentally less frequently (65% vs. 84%, p = .009). No recurrences or ganglioneuroma-specific mortality occurred during follow-up (range, 0-266 months). The systematic review included 14 additional studies and 421 patients. The mean age of diagnosis was 39 years, and 47% were women. AGNs were discovered incidentally in 72% of patients, were predominantly unilateral (99%) and had a mean diameter of 5.8 cm and an unenhanced computed tomography (CT) attenuation of -118 to 49 Hounsfield units (HU). On imaging, 69% of AGNs were homogenous, 41% demonstrated calcifications, and 40% were lobulated. CONCLUSIONS: AGNs are rare benign tumours 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.
Authors: Shawn D St Peter; Patricia A Valusek; Sarah Hill; Mark L Wulkan; Sohail S Shah; Marcello Martinez Ferro; Horatio Bignon; Pablo Laje; Peter A Mattei; Kathleen D Graziano; Oliver J Muensterer; Elizabeth M Pontarelli; Nam X Nguyen; Timothy D Kane; Faisal G Qureshi; Casey M Calkins; Charles M Leys; Joanne E Baerg; George W Holcomb Journal: J Laparoendosc Adv Surg Tech A Date: 2011-07-21 Impact factor: 1.878
Authors: Boris Decarolis; Thorsten Simon; Barbara Krug; Ivo Leuschner; Christian Vokuhl; Peter Kaatsch; Dietrich von Schweinitz; Thomas Klingebiel; Ingo Mueller; Lothar Schweigerer; Frank Berthold; Barbara Hero Journal: BMC Cancer Date: 2016-07-27 Impact factor: 4.430