A total of 109 cases of carcinoma and 5 cases of adenoma of the ampulla of Vater were studied by preparing 5-mm step-wise whole tissue sections in each case. Carcinoma of the ampulla of Vater could be divided into four stages (I, II, III and IV), according to the microscopically verified extent of involvement by the tumor. All 12 patients with a Stage I tumor, defined as one restricted to within the muscle of Oddi as a boundary, showed no lymph node involvement and carried an excellent life expectancy with a relative 5-year survival rate of 85%. Thus, categorization of such a tumor as early carcinoma of the ampulla of Vater is valid. Excluding two unclassifiable cases, the remaining 95 patients with Stage II, III, or IV tumors extending beyond the boundary of Oddi carried relative 5-year survival rates of 11%, 25%, or 24%, respectively. Of the 109 carcinomas, 20 had areas of unequivocal adenoma at the margins, and of the five adenomas, two larger ones had foci of atypical epithelium suggestive of carcinoma in situ. These findings show the close relationship between adenoma and adenocarcinoma of the ampulla of Vater.

https://acsjournals.onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%2819870201%2959%3A3%3C506%3A%3AAID-CNCR2820590326%3E3.0.CO%3B2-%23

Carcinoma of the ampulla of Vater : a clinicopathologic study and pathologic staging of 109 cases of carcinoma and 5 cases of adenoma

The aim of this study was to investigate the differences between the clinicopathological findings in two histologic types of carcinoma of the papilla of Vater. We histologically classified carcinoma of the papilla into two types: 1) an intestinal type that resembles tubular adenocarcinoma of the stomach or colon, and 2) a pancreaticobiliary type that is characterized by papillary projections with scant fibrous cores. We examined 53 cases of resected carcinoma of the papilla. The intestinal-type carcinomas were similar to the intestinal mucosa in that they had lysozyme-containing, Paneth or argyrophil cells, as demonstrated by the immunohistochemically positive stainings for the anti-lysozyme antibody. Although both the sizes of the two types of carcinomas and the age distributions of cases with the two types of carcinoma were almost the same, the prognosis of the cases with the intestinal type was much better than that of the cases with the pancreaticobiliary type. Histological lymph node metastasis was found significantly more often in the pancreaticobiliary type. This result was supported by the fact that small carcinomas of the intestinal type showed little or no invasion into the surrounding interstitium, as opposed to the pancreaticobiliary type, which had a strong infiltrative tendency. The pathogenesis of carcinoma of the papilla of Vater should be further evaluated, taking into consideration the existence of these two histologic types.

Different Clinicopathologic Findings in Two Histologic Types of Carcinoma of Papilla of Vater

Thirty-five consecutive cases of adenocarcinoma of the ampulla of Vater seen over the past 36 years were reviewed. The introduction of new diagnostic techniques over the course of this study improved the accuracy of preoperative diagnosis but did not lead to earlier diagnosis. The surgical resectability rate was 88%, and 53% of postoperative survivors were free of disease at 5 years. Of the 14 patients with metastases to regional lymph nodes, 27% survived disease-free for 5 years. Surgical mortality was 25% for the entire series but has been reduced to 6.6% over the past decade. Surgical mortality was primarily due to leakage of the pancreaticojejunostomy; the risk of pancreaticojejunostomy leak correlated inversely with the degree of chronic pancreatitis in the pancreatic remnant. In 35% of resected cases, a benign adenomatous component was contained within the cancer of the ampulla of Vater. Cure rates are good for this lesion. The most important factor in maximizing cure rate is careful attention to the technical details of pancreaticojejunostomy in order to minimize surgical mortality. Benign adenomas appear to be a frequent precursor of carcinoma of the ampulla of Vater.

Carcinoma of the ampulla of Vater.

Objective To analyze the morbidity and mortality after radical and palliative pancreatic cancer surgery in Norway, especially the risk factors. Summary background data A prospective multicenter study between 1984-1987 including only histologically or cytologically verified adenocarcinoma of the pancreas (N = 442) or the papilla of Vater (N = 30); 84 patients (19%) with pancreatic carcinoma and 24 patients (80%) with papilla carcinoma underwent radical operations. A palliative procedure was performed in 252 patients (53%). Methods Clinical data, surgical procedures and the following morbidity and mortality were recorded on standardized forms. The risk factors were analyzed by a logistic multiple regression model. Results The morbidity, reoperation, and mortality rates were 43, 18, and 11% after radical surgery and 23, 4, and 14% after palliative surgery. Karnofsky's index was the sole independent risk factor for death after radical surgery. Splenectomy, age, and TNM stage influenced morbidity. Diabetes, Karnofsky's index, and liver metastases were risk factors in palliative surgery. Conclusions The morbidity and mortality risks were comparable between total pancreatectomy and a Whipple's procedure and between biliary and a double bypass. Preoperative biliary drainage had no impact on the risks and may be abandoned. High age is a relative and a low Karnofsky's index an absolute contraindication for radical surgery. Nonsurgical palliation of jaundice should be considered according to the presence of independent risk factors.

Morbidity and mortality after radical and palliative pancreatic cancer surgery. Risk factors influencing the short-term results.

In recent years, a number of comprehensive reviews have been written on inherited intestinal polyposis syndromes (1-7), but none has dealt specifically with Gardner's syndrome and none has focused on basic research being carried out in an attempt to understand this syndrome and to improve the medical management of affected patients. A better understanding of this rare genetic disorder is essential for surgeons, gastroenterologists, cancer researchers, and geneticists alike. To the clinician, it poses difficult challenges in management; to the cancer researcher, it presents a rare opportunity to study very early premalignant transformations; and to the geneticist, it poses exciting questions at the cellular, chromosomal, and molecular levels.

Gardner's syndrome. Recent developments in research and management.

Adenocarcinoma of the ampulla of Vater represents about 10% of periampullary neoplasms. This tumor is slow-growing, often amenable to radical surgical resection, and associated with a better prognosis than the other neoplasms which arise in the periampullary area. Correct identification of ampullary carcinoma may be difficult but is essential because of its better prognosis. A review of 31 patients with adenocarcinoma of the ampulla of Vater treated at our institution reveals that the classical history of fluctuating jaundice and the well known findings of a palpable gallbladder and occult blood in the stool may not be present. The treatment of choice for ampullary carcinoma in the absence of hepatic or distant metastases is pancreaticoduodenectomy. Our operative mortality for pancreaticoduodenectomy is 16%, and the five-year survival rate is 32%. The role of palliative procedures, the operative complications, and the survival rates are discussed.

Jimenez-Chapa Jf

Papers

Carcinoma of the ampulla of Vater.