Endocrine Abstracts

Background: Ganglioneuromas are rare benign tumours that arise from neural crest cells including the adrenal medulla. Adrenal ganglioneuromas account for approximately 1% of all adrenal incidentalomas. Due to their rarity, the characterization of these lesions pre- operatively is challenging, with diagnosis typically being made post- operatively on histological assessment.

Method: A retrospective review was performed on the presentation and management of ganglioneuromas at a tertiary referral centre over a 5 year period.

Result: 5 ganglioneuromas were treated, 4 males and 1 female patient. The median age was 23 years (range 16-51 years). The median size radiologically was 5.5 cm (range 3.5-14 cm) and pathologically was 5.8 cm (5-14 cm). All lesions were identified on CT, 3 as incidentalomas and 2 following presentations with abdominal pain. All patients had complete biochemical adrenal work up inclusive of plasma or urinary metanephrines, aldosterone- renin ratio and a 1 mg overnight dexamethasone suppression test. Three patients required additional biochemical assessment including tumour markers. A broad range of radiological investigations were performed to inform management including CT, MRI, MIBG, and MR Angiography. One patient proceeded to an image guided biopsy. All ganglioneuromas were resected with a surgical approach tailored to the lesion. Two underwent open resection, two transperitoneal laparoscopic and one retroperitoneal approach. Four patients had surveillance imaging (CT/ within a year of resection or longer if suspicious findings were identified.

Conclusion: Our experience with ganglioneuromas is of a heterogeneous group with a spectrum of presentations and a resource- intensive approach to reaching a diagnosis. This latter challenge reflects the rarity of the condition.