Duodenal gangliocytic paraganglioma: A case report

Abstract

Introduction: Duodenal gangliocytic paraganglioma (DGP) is a benign tumor that only warrants a surgical resection in the majority of cases. Case Report: We are reporting a case of 42-year-old man who consulted for significant weight loss, fatigue, anemia, and an on–off obstructive jaundice. Imageries showed a 5 cm peri-ampullary duodenal mass. We couldn’t have a diagnosis by endoscopic ultrasound (EUS), so we did a laparoscopic transduodenal tumorectomy and removed the entire mass, which came back as gangliocytic paraganglioma on pathology and immunohistochemistry. Gangliocytic paraganglioma is a benign tumor that most commonly occurs in men and is localized in duodenum. Neuroendocrine tumor, ganglioneuroma, paraganglioma, and schwannoma are the differential diagnoses. Histologic diagnosis is difficult to make. Immunohistochemistry is indispensable for the diagnosis. Often than not, we cannot make diagnosis by a simple biopsy, because the tumor has a submucosal location and three types of cells need to be present for diagnosis: spindle cells, ganglion cells, and epithelial cells. That’s why en-bloc resection of the tumor is frequently needed for accurate diagnosis. Endoscopic resection or laparoscopy is used depending on the characteristics of the tumor. In our case, the 5 cm peri-ampullary tumor warranted a laparoscopic resection. Generally, there is no role for adjuvant therapy in duodenal gangliocytic paraganglioma (DGP). But sometimes, radiotherapy, surgery, somatostatin analog are used for treatment depending on the features of DGP. There is no consensus on follow-up management, but experts agree on the necessity of frequent follow-ups. Conclusion: We believe it is important to include gangliocytic paraganglioma (GP) as a differential diagnosis in patients who present with duodenal masses, weight loss, and general state alteration.