Pancreatic ganglioneuroma: a rare entity with a difficult approach: a case report and systematic review

Abstract

Ganglioneuroma is a very rare benign tumor, arising from neural crest cells in the sympathetic ganglia and adrenal medulla, that is generally located in the retroperitoneum and in the mediastinum; pancreatic involvement is unusual with less than 10 cases reported in the English literature. In this review, we analyzed the English literature published until March 2018, reporting the main features of pancreatic ganglioneuroma. In addition, we report a case of pancreatic ganglioneuroma, with an unknown pre- operative histological diagnosis, affecting a young woman, and treated with surgery. Among 84 identified articles, 79 were excluded and five articles were selected. These reported a total of 5 patients with pancreatic ganglioneuroma, with a median age of 17 years. Selected studies were performed in homogeneous populations. No data were reported about patients’ comorbidity, BMI, perioperative management and long- term outcomes. Due to the lack of specific radiological and cytological features, a correct preoperative diagnosis of ganglioneuroma is very challenging, making surgical resection the main curative option and the most frequent way to make a correct diagnosis.