Large Cell Neuroendocrine Carcinoma of the Ampulla of Vater with Adenocarcinoma and Squamous Cell Carcinoma Components

Abstract

A 73-year-old woman visited our hospital complaining of general fatigue and jaundice. Laboratory tests revealed an elevated total bilirubin, aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and γ-glutamyltransferase. Computed tomography and magnetic resonance imaging demonstrated a mass lesion at the ampulla of Vater with dilatation of the common bile duct and main pancreatic duct. Percutaneous transhepatic cholangiography revealed dilatation of the bile duct and a negative filling defect due to the tumor. Pancreatoduodenectomy was performed. The specimen included an ulcerated firm tumor of the papilla Vater. The surface of the ampulla consisted of well-differentiated papillary adenocarcinoma, whereas the deep layer, such as submucosal or muscular layer, contained large cell neuroendocrine carcinoma and squamous cell carcinoma. Immunohistochemistry revealed that the large cell neuroendocrine carcinoma component was positive for chromogranin A, synaptophysin and CD56. The patient died from multiple liver and bone metastases 13 months after surgery. This is a very rare case of a large cell neuroendocrine carcinoma accompanied by adenocarcinoma and squamous cell carcinoma components.

Report of two cases of large cell neuroendocrine carcinoma of duodenal ampulla with contrasting outcomes following pancreaticoduodenectomy according to the use of adjuvant chemotherapy

“…Our first patient had coexistence of LCNEC and adenoma, and the second patient had coexistence of LCNEC and adenocarcinoma. The associations with NEC and other neoplasms such as adenocarcinoma suggest a common initial carcinogenesis from the same multipoint stem cell [3] , [12] , [17] .…”

“…We report here the case of two patients with LCNEC in the duodenal ampulla of Vater, which were extremely rare malignancies with aggressive behaviour. NET of the ampulla account for 2–8% of ampullary neoplasms, and almost all NET are classified as NET G1 and G2, with NEC being very rare [2] , [3] , [4] , [5] , [6] , [8] , [11] , [12] , [13] . Among 6081 malignant neoplasms of the ampulla, NET was reported in 139 cases in a US population-based study from the National Cancer Institute’s Surveillance, Epidemiology, and End Results data from 1973 to 2006 [2] .…”

“…Further, LCNEC is extremely rare, comprising only 4% (6/139) of the ampullary NET reported in that study. Some individual case reports and small series of LCNEC have been documented [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] . Beggs et al studied a pooled analysis of previously published reports of LCNEC cases in 2012 and collected only 20 patients [8] .…”

“…NEC has been reported often in concomitant observation with adenoma, adenocarcinoma, and other malignancy components [2] , [3] , [8] , [9] , [10] , [11] , [12] . MANEC is classified by WHO when the rate of mixed, adenocarcinoma components is over 30% [1] .…”

“…The prevalence of NET in the ampulla is less than 5% of all duodenal malignancies and only 0.3% of the NET of the entire digestive tract [2] , [3] , [4] , [5] , [6] . Among NET in the ampulla, LCNEC is extremely rare; only a few examples and small case series are documented in the English literature [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] . Because LCNEC of the ampulla is very rare, its clinical features and treatment strategy are not well established.…”