Rare Case of Gangliocytic Paraganglioma

Abstract

Gangliocytic paragangliomas (GP) are very rare tumors, almost exclusively found in duodenum. These are considered benign tumors but rarely may recur or metastasize. The common clinical presentation includes gastrointestinal bleeding, abdominal pain, and in rare cases as obstruction. Definitive treatment is complete resection of tumor, endoscopically or surgically. We present a case of 44-year-old female, who presented with persistent nausea, vomiting, abdominal pain along with on and off melena. She had benign physical exam. Extensive workup done was negative, except CT Enterography showed filling defect. Endoscopy biopsy done showed possible ganglionueroma. She underwent surgery and final pathology results came back as Gangliocytic paraganglioma. Discussion GP is a rare tumor that is located in the duodenum in 90% of cases with a particular predilection to the region of the ampulla of Vater although can be rarely seen in 3rdand 4thportion. Outside duodenum, its occurrence is extremely rare. Due to submucosal nature, exact origin of GP is unknown. The age of presentation is very vast ranging from 15-84 years, with slight male predominance. Clinical presentation is variable, with almost 50% of cases presenting with bleed, followed by anemia and abdominal pain. Rare presentation with duodenal obstruction and biliary obstruction is also seen in some case reports Differentials are related to any mass lesion in duodenum with typical examples of duodenal cancer (benign and malignant) or polyps<./p> Diagnosis is usually made by recognizing the mass according to diagnostic guidelines of presenting case, as in our case of melena and abdominal pain and is confirmed by histopathology. Its main histologic features are epithelioid cells, spindle-shaped cells and ganglion-like cells. Treatment of GPs is resection, endoscopically or surgery, usually depending on size, local invasion and LNs involvement. As most of the GPs are benign, the treatment of choice for gangliocytic paragangliomas is endoscopic resection4. An aggressive behavior may also be suggested by the presence of nuclear pleomorphism, mitotic activity and infiltrative margin. In the case of ampullary paragangliomas, these findings favor radical surgical resection. Reoccurrence rate is very rare but can be seen.2501_A Figure 1. CT abdomen/pelvis with PO/IV Enterography Contrast protocol showing filling defect in periampullary region2501_B Figure 2. high power showing focal aggregates of ganglion cells amidst numerous nerve sheath Schwann cells2501_C Figure 3. low power showing spindle cell tumor underlying the duodenal mucosa