(1/323) Carcinoids of the common bile duct: a case report and literature review.

Carcinoids of the extrahepatic bile ducts and particularly the common bile duct are extremely rare. A 65-year-old woman presented with obstructive jaundice. Laboratory and imaging studies gave results that were consistent with an obstructing lesion in the common bile duct. In this case, a stent was inserted initially to decompress the bile ducts. Subsequently a laparotomy and pancreaticoduodenectomy were performed and a tissue diagnosis of carcinoid of the common bile duct was made. The patient was well with no evidence of recurrence 17 months postoperatively. The authors believe this is the 19th reported case of an extrahepatic bile duct carcinoid.  (+info)

(2/323) Reexploration for periampullary carcinoma: resectability, perioperative results, pathology, and long-term outcome.

OBJECTIVE: This single-institution experience retrospectively reviews the outcomes of patients undergoing reexploration for periampullary carcinoma at a high-volume center. SUMMARY BACKGROUND DATA: Many patients are referred to tertiary centers with periampullary carcinoma after their tumors were deemed unresectable at previous laparotomy. In carefully selected patients, tumor resection is often possible; however, the perioperative results and long-term outcome have not been well defined. METHODS: From November 1991 through December 1997, 78 patients who underwent previous exploratory laparotomy and/or palliative surgery for suspected periampullary carcinoma underwent reexploration. The operative outcome, resectability rate, pathology, and long-term survival rate were compared with 690 concurrent patients who had not undergone previous exploratory surgery. RESULTS: Fifty-two of the 78 patients (67%) undergoing reexploration underwent successful resection by pancreaticoduodenectomy; the remaining 26 patients (34%) were deemed to have unresectable disease. Compared with the 690 patients who had not undergone recent related surgery, the patients in the reoperative group were similar with respect to gender, race, and resectability rate but were significantly younger. The distribution of periampullary cancers by site in the reoperative group undergoing pancreaticoduodenectomy (n = 52) was 60%, 19%, 15%, and 6% for pancreatic, ampullary, distal bile duct, and duodenal tumors, respectively. These figures were similar to the 65%, 14%, 16% and 5% for resectable periampullary cancers found in the primary surgery group (n = 460). Intraoperative blood loss and transfusion requirements did not differ between the two groups. However, the mean operative time was 7.4 hours in the reoperative group, significantly longer than in the control group. On pathologic examination, reoperative patients had smaller tumors, and the percentage of patients with positive lymph nodes in the resection specimen was significantly less. The incidence of positive margins was similar between the two groups. Postoperative lengths of stay, complication rates, and perioperative mortality rates were not higher in reoperative patients. The long-term survival rate was similar between the two resected groups, with a median survival of 24 months in the reoperative group and 20 months in those without previous exploration. CONCLUSIONS: These data demonstrate that patients undergoing reoperation for periampullary carcinoma have similar resectability, perioperative morbidity and mortality, and long-term survival rates as patients undergoing initial exploration. The results suggest that selected patients considered to have unresectable disease at previous surgery should undergo restaging and reexploration at specialized high-volume centers.  (+info)

(3/323) Prognostic value of MIB-1 index and DNA ploidy in resectable ampulla of Vater carcinoma.

OBJECTIVE: To evaluate the prognostic value of the proliferative factors, MIB-1 index, DNA ploidy, and S-phase fraction, and further to determine the independent prognostic factors in ampulla of Vater carcinoma after pancreaticoduodenectomy. SUMMARY BACKGROUND DATA: Cell kinetics are important indicators of the biologic behavior of various human tumors, but only a few authors have reported the application of cell proliferative factors in ampulla of Vater carcinoma. METHODS: Patients undergoing pancreaticoduodenectomy for ampulla of Vater carcinoma were included. Proliferative factors, MIB-1 index, and DNA contents, measured by flow cytometry, were evaluated and compared with the conventional clinicopathologic factors. RESULTS: Ninety resectable ampulla of Vater carcinomas were included. By univariate analysis, MIB-1 index, DNA ploidy, S-phase fraction, stage, and lymph node status were significant prognostic factors. The 5-year survival rate was 40.7% for tumors with MIB-1 index < or =15% and 0% for those with MIB-1 index >15%. Diploid tumors had a significantly better prognosis than aneuploid. Outcomes of stage I and II tumors were more favorable than those of stage III and IV. After multivariate analysis, MIB-1 index, DNA ploidy, and stage remained as the independent prognostic factors. Among the three independent prognostic factors, MIB-1 index was the most powerful. CONCLUSIONS: Both MIB-1 index and DNA ploidy provide important prognostic value and potentially complement the conventional prognostic factors in resectable ampulla of Vater carcinoma. MIB-1 index is the most powerful independent prognostic factor.  (+info)

(4/323) Pancreaticoduodenectomy with or without extended retroperitoneal lymphadenectomy for periampullary adenocarcinoma: comparison of morbidity and mortality and short-term outcome.

OBJECTIVE: This prospective, randomized, single-institution trial was designed to evaluate the end points of mortality, morbidity, and survival in patients undergoing standard versus radical (extended) pancreaticoduodenectomy (including distal gastrectomy and retroperitoneal lymphadenectomy). SUMMARY BACKGROUND DATA: Numerous retrospective reports and one prospective randomized trial have suggested that the performance of an extended lymphadenectomy in association with a pancreaticoduodenal resection may improve long-term survival for some patients with pancreatic and other periampullary adenocarcinomas. Many of these previously published studies can be criticized for their retrospective and nonrandomized designs, for the inclusion of nonconcurrent control groups, and for their small numbers. METHODS: Between April 1996 and December 1997, 114 patients with periampullary adenocarcinoma were enrolled in an ongoing, prospective, randomized trial at The Johns Hopkins Hospital. After intraoperative verification of completely resected periampullary adenocarcinoma, the patients were randomized to receive either a standard pancreaticoduodenectomy (removing only the peripancreatic lymph nodes en bloc with the specimen) or a radical pancreaticoduodenectomy (standard resection plus distal gastrectomy and retroperitoneal lymphadenectomy). All pathology specimens were reviewed and categorized. The postoperative morbidity, mortality, and short-term outcomes were examined. RESULTS: Of the 114 patients randomized, 56 underwent a standard pancreaticoduodenectomy and 58 a radical pancreaticoduodenectomy. The two groups were statistically similar with regard to age and gender, but there was a higher percentage of white patients in the radical group. All the patients in the radical group underwent distal gastric resection, whereas 86% of the patients in the standard group underwent pylorus preservation. The mean operative time in the radical group was 6.8 hours, compared with 6.2 hours in the standard group. There were no significant differences between the two groups with respect to the intraoperative blood loss, transfusion requirements, location of primary tumor, mean tumor size, positive lymph node status, or positive margin status. There were three deaths in the standard group and two in the radical group. The complication rates were 34% for the standard group and 40% for the radical group. Patients undergoing radical resection had a higher incidence of early delayed gastric emptying but had similar rates of other complications, such as pancreatic fistula, wound infection, intraabdominal abscess, and need for reoperation. The mean total number of lymph nodes resected was higher in the radical group. Of the 58 patients in the radical group, only 10% had metastatic carcinoma in the resected retroperitoneal lymph nodes, and none of those patients had the retroperitoneal nodes as the only site of lymph node involvement. The 1-year actuarial survival rate for patients surviving the immediate postoperative periods was 77% for the standard resection group and 83% for the radical resection group. CONCLUSIONS: These data demonstrate that radical pancreaticoduodenectomy (with the addition of a distal gastrectomy and extended retroperitoneal lymphadenectomy to a standard pancreaticoduodenectomy) can be performed with similar morbidity and mortality to standard pancreaticoduodenectomy. However, the survival data are not sufficiently mature and the numbers of patients enrolled are not adequate to allow firm conclusions to be drawn regarding survival benefit.  (+info)

(5/323) Bilateral ovarian carcinoma metastatic from the ampulla of Vater: a rare Krukenberg tumor.

Carcinoma of the ampulla of Vater is a relatively rare neoplasm and its longterm survival rate is considerably high. However, because of differences in tumor pathologic features and local invasiveness, a 5-year survival rate differ widely. We present a case of metastatic carcinoma of the ampulla of Vater presenting as a Krukenberg tumor in a 59-year-old woman. Eight months earlier, she had been diagnosed as well-differentiated adenocarcinoma of the ampulla of Vater. Abdominal examination revealed a hard mass with mild tenderness in the RLQ area. The laboratory findings were unremarkable except for mild anemia. CT scan of the abdomen revealed enlargement of both ovaries. An exploratory laparotomy disclosed bilateral ovarian masses, 18 x 12 x 8 cm and 8 x 5.5 x 4 cm in size, respectively. Histologic findings of the both ovarian masses were consistent with metastatic adenocarcinoma from the ampulla of Vater.  (+info)

(6/323) Germline and somatic mutations of the STK11/LKB1 Peutz-Jeghers gene in pancreatic and biliary cancers.

Peutz-Jeghers syndrome (PJS) is an autosomal-dominant disorder characterized by hamartomatous polyps in the gastrointestinal tract and by pigmented macules of the lips, buccal mucosa, and digits. Less appreciated is the fact that PJS also predisposes patients to an increased risk of gastrointestinal cancer, and pancreatic cancer has been reported in many PJS patients. It was recently shown that germline mutations of the STK11/LKB1 gene are responsible for PJS. We investigated the role of STK11/LKB1 in the development of pancreatic and biliary cancer in patients with and without the PJS. In a PJS patient having a germline splice site mutation in the STK11/LKB1 gene, sequencing analysis of an intestinal polyp and pancreatic cancer from this patient revealed loss of the wild-type allele of the STK11/LKB1 gene in the cancer. Inactivation of STK11/LKB1, by homozygous deletions or somatic sequence mutations coupled with loss of heterozygosity, was also demonstrated in 4-6% of 127 sporadic pancreatic and biliary adenocarcinomas. Our results demonstrate that germline and somatic genetic alterations of the STK11/LKB1 gene may play a causal role in carcinogenesis and that the same gene contributes to the development of both sporadic and familial forms of cancer.  (+info)

(7/323) Adenoma of the ampulla of Vater: a genetic condition?

The etiology of adenoma of the ampulla of Vater is not well understood. Previous authors reported the association of this neoplasm with polycystic kidney disease of two fraternal sisters. They concluded that these two conditions were somehow related. We describe a case of ampullary adenoma associated with polycystic kidney disease. This presentation raises again the question of a possible link between these two diseases.  (+info)

(8/323) Brain metastases from adenoendocrine carcinoma of the common bile duct: a case report.

A 68-year-old man with metastatic brain tumors from adenoendocrine carcinoma of the common bile duct is reported. A common bile duct tumor and a metastatic liver tumor had been resected 6 years and 3 years prior to admission, respectively. Microscopically they showed two components; moderately differentiated tubular adenocarcinoma and neuroendocrine carcinoma. He presented with headache and vomiting and MRI revealed two metastatic brain tumors. They were successfully resected and radiotherapy was carried out. Histological diagnosis of the metastatic brain tumors was neuroendocrine carcinoma, but carbohydrate antigen (CA)-19-9 and carcinoembryonic antigen (CEA)-immunoreactive cells were observed without glandular pattern. Immunohistochemically serotonin and pancreatic polypeptide were detected, but somatostatin was not. As the endocrine cells demonstrated in the normal extrahepatic bile ducts are only somatostatin-containing D cells, these cells are considered to originate as part of a metaplastic process. To our knowledge, this represents the second case of adenoendocrine carcinoma of the common bile duct.  (+info)