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.

Pancreatic Gangliocytic Paraganglioma: A Rare Neuroendocrine Neoplasm

“…3,10 Duodenal GPs are often benign but do offer potential for lymph node metastasis in 5% to 7% cases without any reports of distant metastatic disease and are amenable to less-invasive treatment modalities such as endoscopic resection. 11,12 In contrast, pancreatic GPs are typically larger and may show more aggressive behavior, harboring bone and lymph node metastasis in 1 and 2 reported cases, respectively. Pancreatic GPs, as suggested by the increased propensity for metastasis, may have a greater potential for malignancy.…”

“…Pancreatic GPs may be difficult to distinguish from other pancreatic neuroendocrine neoplasms due to their low incidence, rare presentation, and nonspecific imaging findings. 12,13 The main confounding diagnoses of pancreatic lesions are welldifferentiated NETs, classified as G1, G2, and G3 based on mitotic count and Ki-67 index. Similar to NETs, radiographically, GPs present as a solid mass with homogenous contrast enhancement and occasionally with rich arterial vascularization, which make them difficult to distinguish from other pancreatic NETs or from hypervascularized metastatic lesions, such as from renal cell carcinoma.…”

“…Similar to NETs, radiographically, GPs present as a solid mass with homogenous contrast enhancement and occasionally with rich arterial vascularization, which make them difficult to distinguish from other pancreatic NETs or from hypervascularized metastatic lesions, such as from renal cell carcinoma. 2,6,12 However, when tissue analysis is performed, immunohistochemical staining and morphologic characteristics can distinguish GPs from other pancreatic NETs. 5 Although somatostatin analogs demonstrate benefits in patients with NETs, their use in GPs are unknown.…”

“…However, GPs do have the potential to be functional tumors that exhibit sequelae of catecholamine excess or somatostatin hypersecretion 3,10 . Duodenal GPs are often benign but do offer potential for lymph node metastasis in 5% to 7% cases without any reports of distant metastatic disease and are amenable to less-invasive treatment modalities such as endoscopic resection 11,12 . In contrast, pancreatic GPs are typically larger and may show more aggressive behavior, harboring bone and lymph node metastasis in 1 and 2 reported cases, respectively.…”

“…Pancreatic GPs may be difficult to distinguish from other pancreatic neuroendocrine neoplasms due to their low incidence, rare presentation, and nonspecific imaging findings 12,13 . The main confounding diagnoses of pancreatic lesions are well-differentiated NETs, classified as G1, G2, and G3 based on mitotic count and Ki-67 index.…”