Endocrine Abstracts

Introduction: Ganglioneuroma (GN) is a benign, well-differentiated nerve tumor composed of mature sympathetic ganglion cells and nerve fibers, and most commonly located in the posterior mediastinum and retroperitoneum. GN’s are rarely found in the adrenal gland. This tumor is usually asymptomatic and, in the majority of cases, detected incidentally. The diagnostic confirmation is histologic. We report a case of adrenal GN revealed by an adrenal incidentaloma.

Observation: A 50-year-old man, with no history of comorbidities, who consulted for intense left back pain evolving for 4 months. An abdominal CT scan was performed and showed a left adrenal mass measuring 68*83 mm with a spontaneous density of 37UH. An endocrine workup, including urine catecholamine and cortisol levels and a 1 mg overnight dexamethasone suppression test, was normal. Because of the tumor size, A left adrenalectomy was performed, with no related complication. Histological analysis was in favor of a neuroganglioma, no evidence was found for the malignancy. No recurrence was detected during the follow-up visits.

Discussion: GN is a rare benign tumor of neuroectodermal origin, developed from the sympathetic nervous system. It develops along the sympathetic chains, hence its cervical, mediastinal, retroperitoneal or pelvic localization. Adrenal localization is rare. It is composed of mature ganglion cells and a stroma with nerve cells associated with a schwannian contingent, in contrast to neuroblastoma and ganglioneuroblastoma which are composed of more immature ganglion cells with a higher potential for progression. Ganglioneuroma is most often found in children and young adults. The mode of revelation is often incidental, because retroperitoneal ganglioneuromas are usually asymptomatic. However, sometimes abdominal pain, palpation of an abdominal mass, or compression of nearby organs lead to the diagnosis, as in the case of our patient. The treatment remains surgical and consist in the removal of the tumor. The diagnosis of certainty will only be made after a histological study of the surgical specimen. Local recurrence is exceptional, but the possibility of malignant transformation into a ganglioneuroblastoma is possible, which explains the interest of prolonged surveillance.

Conclusion: Ganglioneuroma may sometimes be similar to other adrenal malignancies. Careful evaluation with endocrine tests and imaging procedures is necessary to provide an accurate diagnosis. Definitive diagnosis can be made by histological examination. The prognosis is very good with surgical removal.