Surgical management of endoscopically unresectable duodenal gangliocytic paraganglioma in a patient with partial upper gastrointestinal obstruction

Gangliocytic paragangliomas are unusual and often misunderstood tumors that occur almost exclusively in the second portion of the duodenum, although they have been described in other sites such as the urinary bladder, spermatic cord, prostate, urethra, uterus and scalp. We describe our experience with the surgical management of an endoscopically unresectable gangliocytic paraganglioma located in the third part of the duodenum causing a partial upper gastrointestinal obstruction. A fifty-two-year-old male presented to the Geisinger clinic with a four-year history of postprandial projectile vomiting associated with epigastric discomfort. Computed tomography scan revealed an oval-shaped filling defect in the third part of the duodenum. Endoscopic ultrasonography showed a 22 × 16 × 35 mm submucosal mass that was not amenable to an endoscopic resection. Exploratory laparotomy revealed an absence of extraduodenal involvement. A long-stalked tumor was successfully excised and extruded through a longitudinal duodenotomy. The pathology report showed a gangliocytic paraganglioma with negative lymph nodes. In patients presenting with prolonged recurrent attacks of vomiting, diagnostic workup to exclude anatomic causes is mandatory. Gangliocytic paraganglioma must be considered in the differential diagnosis of an intraduodenal tumor.