Duodenal gangliocytic paraganglioma with lymph node metastasis: A systematic case review

Abstract

Introduction: Duodenal gangliocytic paragangliomas (DGPs) are rare tumors, most commonly located in the 2nd portion of the duodenum. Their origin is poorly understood and management is uncertain. Typically benign, they infrequently metastasize to lymph node and distant sites. Objective: A systematic literature search for DGPs with lymph node metastases was performed. Epidemiological, diagnostic, management, surveillance and outcome data were recorded. The histopathology and immunohistochemistry of these tumors and possible predictors of lymph node metastases were revisited. Thirty three cases of DGPs with lymph node metastases were included. Mean patient age was 48 years, with no predilection for sex. Discussion: Presenting complaints included abdominal discomfort and gastrointestinal bleeding/anemia. Tumor size (maximum diameter) ranged from 1 to 9 cm, with a mean Luke Hartford1, Alexsi Sherazadishvili2, Ken Leslie3 Affiliations: 1General Surgery Resident, Department of General Surgery, London Health Sciences Centre, 800 Commissioners Road East, Room E2-213, Zone E, London, Ontario, Canada; 2MD Candidate, Class of 2019, Schulich School of Medicine and Dentistry, University of Western Ontario, 1151 Richmond St. London, Ontario, Canada; 3Chair/ Chief Division of General Surgery, Associate Professor, London Health Sciences Centre, 800 Commissioners Road East, Room E2-213, Zone E, London, Ontario, Canada. Corresponding Author: Luke Hartford, General Surgery Resident, Department of General Surgery, London Health Sciences Centre, 800 Commissioners Road East, Room E2-213, Zone E, London, Ontario, Canada, N6A 5A5, Email: luke.hartford@lhsc.on.ca Received: 14 October 2017 Accepted: 25 November 2017 Published: 21 December 2017 of 3.1 cm. Serum/urine tumor markers and hormones were inconsistent. However, there was evidence of neuroendocrine activity. Esophagogastroduodenoscopy successfully identified the lesion in 23/23 reported cases, but had no role in tissue diagnosis. Computed tomography scan and endoscopic ultrasound correctly identified a mass in 84% and 100% of reported cases and were successful in detecting lymph node involvement in 47% and 64% of cases. Pancreatoduodenectomy was the definitive treatment in 87% of the cases. Possible predictors of lymph node metastasis included tumor extension and angiolymphatic invasion, as well as changes noted in immunohistochemistry. Conclusion: We suggest diagnostic aids, management and surveillance for DGPs with lymph node metastases. Due to uncertain malignant potential, surgical management and treatment adjuncts like chemotherapy and radiation should be investigated. Surveillance has not been well established and should be focused on diagnostic imaging and close clinical follow-up.