Massive retroperiteoneal ganglioneuroma presenting with small bowel obstruction 18 years following initial diagnosis

Background: Ganglioneuroma is a rare tumour of neural crest origin, which arises from maturation of a neuroblastoma. While previously considered to be non-functioning, they are now known to be frequently endocrinologically active. Aims and methods: We report a case of a massive retroperitoneal ganglioneuroma presenting with small bowel obstruction in an adult, 18 years after initial diagnosis. Urinary dopamine levels were elevated, but other catecholamines were within normal limits. This is the first report in the English-language literature of a retroperitoneal ganglioneuroma presenting with or causing intestinal obstruction. We also review the metabolic, radiological, and histological features of these tumours. Relevant publications were identified from a Medline search using the MeSH headings 'ganglioneuroma','retroperitoneal neoplasms' and 'intestinal obstruction', and also from the reference lists of retrieved articles. Conclusions: Ganglioneuroma can grow to a massive size and present in a varied manner. It should be included in the differential diagnosis of any large retroperitoneal or mediastinal mass, including those causing bowel obstruction.