Villous tumors of the ampulla of vater are rare tumors and are premalignant. A large percentage of them harbor malignant foci at the time of diagnosis. Presenting a case where we have observed the adenoma carcinoma sequence. A 65-year-old patient presented with vague pain in the right hypochondrium for 2 years. Patient presented with a duodenoscopy report showing a large benign-appearing villous tumor at the ampulla. Endoscopic biopsies proved it to be a benign villous adenoma. Being otherwise asymptomatic, the patient refused treatment and was later lost to follow-up. She later followed up after 18 months with obstructive jaundice, and the endoscopy at this time showed changes suggestive of malignant transformation in the tumor, which was confirmed by biopsy. After investigations, she was subjected to Whipple's pancreatico-duodenectomy. Adenoma-carcinoma sequence of colonic adenomas has been well documented. The same analogy has been applied to ampullary adenomas. A review of literature shows that a high percentage of these tumors have malignant foci, hence complete removal with thorough histopathological evaluation is necessary. Although size has no relation with the incidence of malignancy, the bigger the size the more likelihood there is of getting non-representative endoscopic sampling. If endoscopic biopsies are negative, a frozen section of the tissue is a must before deciding the final surgical treatment. Various treatment options are available for benign adenomas. For malignant tumors pancreaticoduodenectomy is advised.
Villous tumors of the ampulla of vater are rare (<1% of duodenal neoplasms); giant villous adenomas (>3 centimeters) are even rarer . These are considered to be premalignant lesions  .The incidence of malignancy at the time of diagnosis varies from 26% to 50% in various series  . An analogy is often drawn between the adenoma-carcinoma sequence for colonic adenoma and ampullary adenoma and carcinoma . Presenting herewith a case where a morphological and histological adenoma turned into an obvious carcinoma due to the patient's reluctance for treatment.
A 65-year-old anicteric female patient presented with vague pain in the right hypochondrium of 2 years' duration. The patient had undergone cholecystectomy for gallstone disease 6 months previously. She presented to us with a duodenoscopy report suggestive of a large benign-appearing villous tumor at the ampulla. (Fig.1).
Endoscopic biopsies proved it to be a benign villous adenoma. Biochemical and radiological investigations at that time did not reveal any biliary obstruction. The patient was offered resectional treatment in view of the large size of the tumor and possibility of harboring malignancy. The patient refused treatment and was lost to follow-up thereafter.
She followed up 18 months later with obstructive jaundice and the lateral viewing endoscopy at this time showed changes in the tumor, which were suggestive of a malignant transformation. (Fig.2)
Repeat endoscopic biopsy revealed a well-differentiated adenocarcinoma. Computed Tomography (CT) scan was performed to look for vascular involvement. This revealed a double duct sign and no evidence of unresectability (Fig.3).
The patient was subjected to Whipple's pancreatico-duodenectomy. The postoperative course was uneventful and the patient followed up for a period of 24 months during which she was asymptomatic.
Adenoma-carcinoma sequence of colonic adenomas has been postulated and well documented . The same analogy has been applied to ampullary adenomas. These lesions are considered premalignant and excluding malignant disease preoperatively is not always possible . A review of literature shows that a high percentage of these tumors have focus of malignancy and hence complete removal with thorough histopathological evaluation is necessary . If endoscopic biopsies are negative, a frozen section of the tissue is a must before deciding the final surgical treatment. For smaller and benign lesions, various treatment options are described viz. transduodenal excision , endoscopic excision , pancreas preserving segmental resection of the duodenum  and even laparoscopic transduodenal excision with biliary reconstruction . Pylorus preserving or classical Whipple's pancreatico-duodenectomy is recommended for lesions which are large in size or are proven to be malignant . Pancreatico-duodenectomy is also recommended for all good risk patients in whom there is a doubt of benignity of the lesion  .
This article was first published on GastroHep.com in 2007.
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This article was first published on GastroHep in February 2007.