Gangliocytic Paraganglioma with Carcinoma of the Ampulla of Vater

Abstract

The patient was a "73" -year-old woman who visited our hospital with the chief complaint of weight loss. Upper gastrointestinal endoscopy revealed an enlarged ampulla of Vater, and a biopsy led to a diagnosis of Group "4" gastric carcinoma; suspicious of adenocarcinoma. There were no findings suggesting invasion into the muscle layer of duodenum, despite tumor mass formation being observed in the sphincter of Oddi. We performed endoscopic papillectomy for both diagnostic and therapeutic purposes. Pathologically, a well-differentiated adenocarcinoma existed in the superficial layer of the mucous membrane of the papilla of Vater, and gangliocytic paraganglioma was present in the deep portion. The resected margins of both lesions were negative.

Keywords: ampulla of Vater; carcinoma; endoscopic papillectomy; gangliocytic paraganglioma.

Figure 1.

Endoscopic image of the papilla of Vater. The papilla of Vater was slightly tense and swollen, and the oral side was slightly reddish. After applying indigo carmine, erythema was seen on the oral side of the papilla of Vater.

Figure 2.

CT image (non-contrast-enhanced CT) of the papilla of Vater. An enlarged overhanging papilla of Vater was observed in the duodenum (arrow).

Figure 3.

EUS images of the papilla of Vater. A homogeneous and hypoechoic tumor mass, measuring 12-mm in maximum diameter, was observed (arrow) and the muscle layer of the duodenum was maintained (arrowhead). The muscle of the papilla of Vater was preserved and no tumor progression was evident in either the bile duct (*) or the pancreatic duct (**).

Figure 4.

ERCP images. No dilation was observed in either the bile duct or the pancreatic duct. IDUS, similarly, revealed no evidence of tumor progression in either the bile duct or the pancreatic duct.

Figure 5.

Procedure of endoscopic papillectomy. a: The snare was squeezed around the target in the direction from the oral side to the anal side. b: After resection, the openings of the bile duct (arrow) and the pancreatic duct (arrowhead) could be confirmed. c: The frenulum was clipped for closure. d: A stent was placed in the bile duct and the pancreatic duct. e: The resected specimen: Macroscopically, an en bloc resection was achieved.

Figure 6.

Pathological images of the resected specimen in Hematoxylin and Eosin staining. a: A loupe image of the cancer site, b: Magnification of the outlined square showed the dotted line in a. There was growth of the mucosal epithelium with an obvious stacking of nuclei. Most of the specimen showed cytological atypia corresponding to adenoma, c: Magnification of the outlined square showed the solid line in a. There were some strongly atypical gland ducts, which were consistent with adenocarcinoma. d: A loupe image of gangliocytic paraganglioma, e: Magnification of the outlined square showed the solid line in d: Cell clumps proliferating to form folliculi (*) were seen. f: Magnification of the outlined square dotted line in e. Bar=3 mm.

Figure 7.

Immunostaining of gangliocytic paraganglioma. Immunostaining results were AE1AE3-positive, CD56-positive, Chromogranin A-negative, NSE-positive, S100-positive (Magnification of the outlined square), and Synaptophysin-positive, leading to the diagnosis of GP. Bar=150 μm.