Signet ring cell carcinoma of the ampulla of Vater is extremely rare and only 12 cases have previously been described in the English language literature. We report a case of advanced signet ring cell carcinoma of the ampulla of Vater, with invasion of the duodenum and pancreas.
A 59-year-old man presented at a local hospital with upper abdominal pain and icterus. Obstructive jaundice was diagnosed and he was referred to our hospital. Abdominal computed tomography showed dilatation of the common bile duct and the pancreatic duct. Duodenoscopy indicated an irregularly shaped erosion on the ampulla of Vater. Histological examination of a biopsy revealed adenocarcinoma. Duodenography revealed irregularity of the second portion of the duodenum wall. The diagnosis was carcinoma of the ampulla of Vater, and a pancreaticoduodenectomy was performed. The histopathological findings detected signet ring cell carcinoma.
Although several cases have been reported, the detailed clinicopathological features and prognosis are not clear. Additional reports are warranted.
"The 5-year survival rate for gastric signet ring carcinoma is 16.2%. Although the previous cases did not have long-term follow up but findings are suggested that signet ring carcinoma of ampulla of Vater has better prognosis. "
[Show abstract][Hide abstract] ABSTRACT: Signet ring carcinoma is a common type of adenocarcinoma of stomach but its occurrence in ampulla of Vater is extremely rare. There are only a few previous reported cases of signet ring carcinoma of ampulla of Vater. Here we reported a 61-year-old woman with obstructive jaundice. Ultrasonography and computed tomography (CT scan) examination showed intra- and extrahepatic bile duct dilatation. Endoscopic examination with biopsies revealed a small-size mass in ampulla of Vater with diagnosis of signet ring carcinoma. On consequent pancreatoduodenectomy the tumor was diagnosed as T2N0M0, stage IB. Because of the specific site of signet ring carcinoma of ampulla of Vater, the tumor seems to present itself at an early stage of disease. We review in the literature to suggest our idea.
"Two possible theories have been proposed: the presence of gastric heterotopia in the ampulla of Vater or the existence of a perivaterian duodenal heterotopia of ulcerous etiology as the origin of a signet ring cell tumor which secondarily invades the ampulla of Vater . Akatsu et al.  have summarized the previous 14 cases (eight men and six women) and concluded that the median age at diagnosis was 57 years (range, 32–83 years), approximately 15 years older than SRCC of the stomach, but similar to the median age for SRCC of the large bowel. Hara et al.  report the case of seven patients presented SRCC of the Vater's ampulla. "
[Show abstract][Hide abstract] ABSTRACT: Most tumors affecting Vater's ampulla are adenocarcinomas, and other histological variants are less frequent. Signet ring cell carcinoma is more commonly found in the stomach than at other sites of the digestive system. Signet ring cell carcinoma of the Vater's ampulla is extremely rare, and only 15 cases have previously been described in the literature. It mainly occurs in elderly patients (median age = 57 years). We report a case of advanced signet ring cell carcinoma of the ampulla of Vater, with invasion of the duodenum (D3) admitted in the Medical Oncology Unit of HASSAN II University Hospital.
Case Reports in Oncological Medicine 09/2012; 2012:402798. DOI:10.1155/2012/402798
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