S6118 Case of a Gangliocytic Paraganglioma of the Duodenum Presenting as Isolated Weight Loss: Diagnostic Challenges and Management Highlights

Introduction: Gangliocytic paraganglioma (GP) is a rare tumor that commonly involves the gastrointestinal system and often occurs in the second portion of the duodenum. Aside from gastrointestinal involvement, some case reports present GP of the lungs, heart, head and neck. GP is characterized by its triphasic cellular differentiation on histopathology. Most GPs are considered benign and are amenable to excision, however, periampullary GPs should be considered as a tumor with malignant potential. Case Description/Methods: This is the case of a 73-year-old woman presenting for an unintentional 10-pound weight loss over the previous year and a computed tomography scan of the abdomen and pelvis showing a mass in the second portion of the duodenum with omental nodules. She had no other symptoms. An upper endoscopy revealed a pedunculated 5 cm polypoid mass that was endoscopically resected and found to be a gangliocytic paraganglioma on pathology. GP is the third most frequent histopathologic type of gastrointestinal neuroendocrine tumors (NET) after gastrinomas and somatostatinomas. Clinical presentations vary from asymptomatic patients to others having gastrointestinal bleeding, melena, anemia and abdominal pain. GP presents as a single solid well-demarcated mass that is polypoid, pedunculated or sessile. Histological features of GP show 3 cell types: spindle cells, ganglion or ganglion-like cells, and epithelioid cells. Biopsy specimens might not contain all 3 characteristic cell types, thus definitive diagnosis could be challenging. Periampullary GP should be considered as a tumor with malignant potential. The optimal treatment has not been clarified, but endoscopic resection remains the most common treatment of choice. Discussion: This case underscores the importance of including rare tumors with malignant potential, such as gangliocytic paraganglioma (GP), in the differential diagnosis of duodenal masses. Histologic features, including spindle, ganglion, or epithelioid cells, combined with characteristic immunohistochemical staining, are essential for confirmation. Treatment typically involves endoscopic or surgical resection, depending on the depth of invasion. Further longitudinal studies are needed to establish standardized management protocols and enhance patient care.