Gangliocytic paragangliomas (GP) are a rare tumour, most commonly located in the 2nd portion of the duodenum. Their origin is poorly understood and management is uncertain. Typically exhibiting benign behaviour, they infrequently metastasize to lymph nodes (LN) and distant sites. A systematic literature search for duodenal gangliocytic paragangliomas (DPGs) with LN metastases was performed. Epidemiological,  diagnostic, management, surveillance and outcome data were recorded. The histopathology and immunohistochemistry (IHC) of these tumours and possible predictors of LN metastases were revisited. A total of 33 cases of duodenal GP with LN metastases were included. The mean age of patients was 48 years (SD 15.3 years), with no predilection for sex. The 2nd portion of the duodenum was the most common location and presenting complaints included abdominal discomfort and GI bleeding/anemia most frequently. Tumour size (maximum diameter) ranged from 1 to 9 cm, with a mean of 3.1 cm, which were larger than DGPs without LN metastases (2.5 cm). Serum/urine tumour markers and hormones were inconsistent; however, there was evidence of neuroendocrine activity. Esophagogastroduodenoscopy (EGD) successfully identified the lesion in 23/23 reported cases, but had no role in tissue diagnosis. CT and endoscopic ultrasound (EUS) correctly identified a mass in 84% and 100% of reported cases and were successful in detecting LN involvement in 47% and 64% of cases. Pancreatoduodenectomy (PD) was the definitive treatment in 87% of the cases. There was 1 patient mortality during initial hospital stay. One-year mortality was 4.3%; Five-year mortality was 25%, accounting for length of follow-up reported. Possible predictors of LN metastasis included tumour extension and angiolymphatic invasion, as well as changes noted in IHC. We suggest diagnostic aids, management and surveillance for DGPs with LN metastases. Treating clinicians should be aware of the uncertain malignant potential and have an informed discussion with their patients as to surgical management and treatment adjuncts such as chemotherapy and radiation therapy, taking into consideration patient comorbidities, risks/benefits and patient preference. Surveillance has not been well established and should be focused on diagnostic imaging and close clinical follow-up.

Duodenal Gangliocytic Paraganglioma with Lymph Node Metastasis: A Systematic Case Review of Epidemiology, Diagnosis, Surgical Management, Surveillance, Histopathology and Outcomes