Duodenal gangliocytic paraganglioma: a rare case report

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Abstract

Gangliocytic paraganglioma (GP) is a rare tumor that involves the gastrointestinal system and often occurs in the second portion of the duodenum. This is the case of a 73-year-old female presenting for an unintentional 10-pound weight loss over the previous year and a computed tomography (CT) 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 GP 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 those 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 three 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. En bloc resection with negative margins for a pedunculated lesion is possible. This is a rare case of GP in a female patient presenting with weight loss only. It highlights the importance of considering GP in the differential diagnosis for duodenal masses, while keeping a broad differential due to the low rates of diagnostic biopsies. More research is needed to establish standardized management protocols for GP and improve patient care.

Keywords

gangliocytic paraganglioma / duodenal mass / endoscopic resection / case report

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