A Case of Concurrent Ampullary Adenoma and Gangliocytic Paraganglioma at the Minor Papilla Treated with Endoscopic Resection

Abstract

A gangliocytic paraganglioma is a benign tumor of the digestive system with a very low incidence. The tumor is histopathologically characterized by a triphasic pattern consisting of epithelioid, ganglion, and spindle-shaped Schwann cells. In most cases, it occurs in the second portion of the duodenum near the ampulla of Vater. We report a case of a gangliocytic paraganglioma occurring at the minor duodenal papilla (a rare location) with a concurrent adenoma of the ampulla of Vater. Both lesions were treated simultaneously using endoscopic resection. Additionally, we have presented a literature review.

Keywords: Adenoma; Ampulla of Vater; Endoscopic mucosal resection; Paraganglioma.

Fig. 1.

(A) Computed tomography scan showing no demonstrable mass involving the ampulla of Vater. No definitive evidence of bile or pancreatic ductal dilatation is observed. (B) Duodenoscopic image showing a discolored and partially reddish appearing adenoma of the ampulla of Vater. (C) Image showing endoscopic snare papillectomy performed after saline injection. A hemoclip is applied to close the papillectomy site. (D) Image showing selective cannulation of the pancreatic duct and the insertion of a plastic pancreatic stent (single pigtail, 5-Fr × 3 cm) into the pancreatic duct for prevention of pancreatitis.

Fig. 2.

(A) Duodenoscopic image showing a subepithelial tumor measuring approximately 2 cm in size, involving the minor papilla. (B, C) Image showing endoscopic mucosal resection performed after saline injection. Mild oozing bleeding is observed, and 2 hemoclips were used to control bleeding.

Fig. 3.

(A) Post-papillectomy image showing a well-demarcated, elevated firm lesion measuring 1.7×1.5×0.3 cm in size. (B) Histological section (hematoxylin and eosin [H&E], ×40) showing dysplastic glandular cells clustered on the duodenal papillary surface. (C) Non-neoplastic mucosa adjacent to the tumor shows a mixture of irregularly oriented smooth muscle bundles and non-neoplastic biliary glands, suggestive of duodenal papillary involvement (H&E, ×40). (D) Dysplastic glandular cells show enlarged, elongated hyperchromatic nuclei of uniform size, with no loss of polarity, comparable to low-grade dysplasia (H&E, ×400).

Fig. 4.

(A) Image obtained after endoscopic mucosal resection showing a well-demarcated, elevated firm lesion measuring 1.9×1.3×0.6 cm. (B) Histological section (hematoxylin and eosin [H&E], ×20) showing the tumor (left) and adjacent non-neoplastic duodenal mucosa. The tumor is a well-demarcated, lobulated submucosal lesion. (C) The tumor is heterogeneous in nature and shows 3 components as follows: Schwann cells, ganglion cells, and neuroendocrine cells (H&E, ×40). (D) Neuroendocrine cells with a trabeculated pattern can be observed (H&E, ×100). (E) Ganglion cells and polygonal cells with large round nuclei and small nucleoli and finely dispersed chromatin can be observed (H&E, ×400). (F) Spindle-shaped Schwann cells with wavy and tapered nuclei can be observed (H&E, ×400).

Fig. 5.

(A) Image showing immunohistochemical staining (×200) with Schwann cells and ganglion cells stained positive for S-100 protein. (B) Neuroendocrine and ganglion cells are stained positive for synaptophysin (×200). (C) Neuroendocrine cells are focally and weakly positive for chromogranin stain (×200). (D) Ki-67 staining (×200) shows a low proliferation index (<1%).