Factors predictive of survival in ampullary carcinoma.

OBJECTIVE: To review the recent Memorial Sloan-Kettering Cancer Center experience with adenocarcinoma of the ampulla of Vater and to identify clinicopathologic factors that have an impact on patient survival. SUMMARY BACKGROUND DATA: The prognosis for patients with tumors of the ampulla of Vater is improved relative to other periampullary neoplasms. Identification of independent prognostic factors in ampullary tumors has been limited by small numbers of tumors and a lack of pathologic review. METHODS: Data were collected prospectively for patients presenting with periampullary carcinomas to the Memorial Sloan-Kettering Cancer Center between October 15, 1983 and June 30, 1995. The correlation between clinicopathologic variables and survival of ampullary carcinoma was tested by the Kaplan-Meier method and log-rank test, and Cox proportional hazards regression. Survival of patients with periampullary adenocarcinomas was compared by the Kaplan-Meier method. RESULTS: In 123 patients presenting with ampullary carcinoma, 101 tumors (82.1%) were resected. Factors significantly correlated with improved survival were resection (p < 0.01), and in resected tumors, negative nodes (p = 0.04) and margins (p = 0.02) independently predicted for improved survival. In periampullary tumors, the highest rates of resection and overall survival (median, 43.6 months) were found in ampullary carcinomas. CONCLUSIONS: Factors predictive of improved survival in ampullary carcinoma include resection, negative margins, and negative nodes. Improved overall survival in ampullary relative to periampullary adenocarcinoma is due in part to a significantly higher rate of resection.     

Radical resection in signet ring carcinoma of the ampulla of Vater: report of an 11-year survivor

Carcinomas of the ampulla of Vater are uncommon tumors but have a better prognosis than typical pancreatic cancers. They tend to be well or moderately differentiated while the poorly differentiated variants are still quite aggressive. Signet ring features associated with poor differentiation traditionally confer a dismal prognosis in other gastrointestinal malignancies, particularly gastric cancer. Signet cell ring morphology has only been described in a few ampullary carcinomas with all cases reporting short follow-up. We describe a 58-year-old woman who presented with painless jaundice and a prominent ampulla of Vater by endoscopy. She underwent pancreaticoduodenectomy and was found to have a 1.0 x 0.8 cm poorly differentiated carcinoma with prominent signet ring cell features arising from the ampulla of Vater and invading into the periampullary duodenum but sparing the pancreatic parenchyma. No distant or nodal metastases were seen (pT2N0M0). No adjuvant therapy was given, and the patient remains free of disease with 134 months of follow-up.     

Carcinoma of the pancreas and periampullary region: a 41 year experience.

A retrospective review of 329 cases of adenocarcinoma of the pancreas and 31 adenocarcinomas of the ampulla and and common bile duct seen between the years 1929 and 1973 was carried out. The most common complaints for carcinoma of the pancreas were pain, weight loss, and jaundice in that order of frequency; while jaundice was the most common complaint with periampullary lesions. The most common procedure carried out was a gastric and/or biliary bypass. Thirty-five patients underwent pancreatoduodenectomy. The survival of this latter group was longer and better than those undergoing bypass and in 40% of patients with ampullary carcinoma a cure was effected. Patients undergoing bypass did not live longer than patients undergoing simple exploratory laparotomy. Duration of symptoms and location of tumor within the pancreas (excluding ampullary tumors) did not appear significantly to alter the prognosis. In view of our experience it is felt that pancreatoduodenectomy should be undertaken whenever the tumor is deemed resectable as this provides the only chance for cure and the best palliation.     

Periampullary choledochoduodenal fistula in ampullary carcinoma

Most patients with ampullary carcinoma have obstructive jaundice without cholangitis. We experienced a patient with ampullary carcinoma who presented with obstructive jaundice and cholangitis, probably because of an accompanying periampullary choledochoduodenal fistula. A 77-year-old Japanese man had jaundice, high fever, and upper abdominal pain and was diagnosed, at another hospital, with obstructive cholangitis. On admission to our hospital, his symptoms and signs had subsided spontaneously. Abdominal ultrasonography showed cholecystolithiasis and dilatation of the common bile duct. Duodenoscopy showed an ulcerating tumor at the oral prominence of the ampulla of Vater and a periampullary choledochoduodenal fistula at the bottom of the ulcer. Biopsy from the fistula showed well differentiated adenocarcinoma. With a diagnosis of ampullary carcinoma with fistula formation, the patient underwent pylorus-preserving pancreatoduodenectomy. The diagnosis was confirmed by histology. This communication presents a unique case of ampullary carcinoma that caused obstructive jaundice, which subsided spontaneously but was associated with cholangitis caused by the divergent effects of the periampullary choledochoduodenal fistula formed by the carcinoma. Received: September 5, 2000 / Accepted: December 22, 2000     

Results of surgical treatment of periampullary tumors: a thirty-five-year experience

Data on 126 consecutive patients with periampullary tumors resected at the Cleveland Clinic between January 1950 and December 1984 were reviewed. One hundred five patients underwent pancreatoduodenal resection, 10 patients total pancreatectomy, and 11 patients local resection of the tumor. The site of tumor was ampulla of Vater (59), head of the pancreas (30), duodenum (20), and distal common bile duct (11). Six patients had benign disease. The operative mortality rate for radical resection for the entire period was 7.8%; it has declined to 5.4% since 1974. The operative mortality rate for local resection was 9.1% (one patient). The overall 5-year survival rate for all malignant tumors of the periampullary area was 28% and 25.5% for invasive adenocarcinoma. Survival was affected primarily by location and histologic findings. The 5-year survival rate for adenocarcinoma of the ampulla of Vater was 37.2%, 27.5% for the duodenum, 16.7% for the distal common bile, and 4.3% for the pancreas (p = 0.0001). Papillary adenocarcinoma had a 5-year survival rate of 49.2% in contrast to 18.4% for nonpapillary ductal adenocarcinoma (p = 0.002). Patients with ampullary adenocarcinoma treated by local resection had a 5-year survival rate of 40.9%. These data justify continued use of a selective radical approach in the resection of most periampullary tumors with local resection for small tumors in high-risk patients.     

Carcinogenesis of cancer of the papilla and ampulla: pathophysiological facts and molecular biological mechanisms

BACKGROUND: Ampullary cancer has one of the highest resectability rates and best prognoses among neoplasms arising in the periampullary region. DISCUSSION: Early diagnosis due to early symptoms can partially explain the better prognosis as compared to other cancers of the periampullary region, but biologic factors should also be taken in account. In the past few years, the molecular mechanisms underlying this disease have been investigated and alterations of genes that regulate different cell functions have been described. Mutations of K-ras and of the tumor suppressor genes APC, p16 and p53 indicate a major disturbance in cell cycle regulation. CONCLUSIONS: If the molecular profile of ampullary cancer is examined in terms of rate and type of molecular changes, it seems to be more similar to intestinal than to pancreatic cancer. Furthermore, the fact that many ampullary carcinomas arise from adenomas and the frequent finding of ampullary tumors in patients affected by polyposis syndromes also suggest that ampullary and colon cancers share common molecular mechanisms of carcinogenesis.     

Carcinoma of the Ampulla of Vater: Factors Influencing Long-term Survival of 127 Patients with Resection

INTRODUCTION: The prognosis for patients with carcinoma of the ampulla of Vater is improved relative to other periampullary neoplasms. Identification of independent prognostic factors in ampullary carcinomas has been limited by the small number of tumors resected. The aim of the present study was to determine the clinicopathologic factors that influence long-term survival in patients with resected ampullary carcinoma. METHODS: Clinicopathologic data were retrospectively reviewed for patients with ampullary carcinomas radically resected between March 1987 and September 2002. The correlation between clinicopathologic variables and survival of patients after resection was examined by the Kaplan-Meier method, the log-rank test, and Cox proportional hazards regression. Ampullary carcinomas were radically resected in 127 patients either by pancreaticoduodenectomy (n = 124) or local resection (n = 3). RESULTS: Hospital mortality was 9.7%. The overall actuarial survival rates (including hospital deaths) at 1, 3, 5, and 10 years were 76.2%, 46.8%, 43.3%, and 35.7%, respectively. Factors that significantly influenced survival were lymph node status (P < 0.001), depth of tumor infiltration (P = 0.029), and TNM stage (P < 0.001) on univariate analysis. On multivariate analysis, both depth of infiltration and lymph node status were the independent determinants of survival after resection (P = 0.003, P = 0.005, respectively). CONCLUSIONS: Carcinoma of the ampulla of Vater has a higher resectability rate and a much better survival rate than pancreatic cancer. Pancreaticoduodenectomy is the treatment of choice for this tumor. Long-term survival was independently influenced by the depth of tumor infiltration and lymph node metastasis.     

High-grade neuroendocrine carcinoma of the ampulla of vater: a clinicopathologic and immunohistochemical analysis of 14 cases

We describe the clinical and pathologic features of 14 cases of high-grade neuroendocrine carcinoma (HGNEC) of the ampulla of Vater classified according to WHO classification of lung tumors into small cell carcinoma (SCC, 6 cases) and large cell neuroendocrine carcinoma (LCNEC, 8 cases) types. The immunohistochemical findings were compared with those of 13 cases of primary poorly differentiated ampullary adenocarcinomas (PDACA) lacking neuroendocrine morphology. The mean age of 10 males and 4 females was 70 years. The mean tumor size was 2.5 cm. Ten of 13 patients had lymph node metastases (mean, 2.3 nodes involved). Documented sites of distant metastases included brain and liver. Overall, 64% of patients with ampullary HGNEC died of disease (mean follow-up, 14.5 months). Four patients had no evidence of disease after resection (mean, 20 months). Half of the tumors were associated with adenomas of the adjacent mucosa, 2 with high-grade dysplasia. Two HGNECs were combined with a conventional adenocarcinoma and another with a squamous cell carcinoma component. By immunohistochemistry, the HGNECs were positive for cytokeratins (AE1/AE3, 100%; Cam5.2, 67%; CK7, 87%; CK20, 38%), similar to the pattern found in PDACAs. p27 expression was lost in 1 case of HGNEC and in all PDACAs. Retinoblastoma (Rb) protein expression was lost in 60% of HGNECs and in none of the PDACA cases. In conclusion, HGNECs of the ampulla are rare (2%-3% of ampullary tumors in our material). The clinical course parallels that of their pulmonary counterparts and appears to be worse than that of locally advanced ampullary adenocarcinomas. The association with adenoma and or conventional adenocarcinoma components may suggest a common pathway in the initial carcinogenesis of these two types of tumors. Loss of Rb expression, a characteristic finding in pulmonary SCCs, is present in almost half of ampullary HGNECs. In contrast, p27 expression is lost in PDACAs and retained in most HGNECs. Thus, there are differences in the molecular phenotypes of these two types of ampullary carcinoma, supporting the distinction of poorly differentiated carcinomas with a neuroendocrine phenotype from those without.     

Significance of tumor spread in adenocarcinoma of the ampulla of Vater

Twenty-eight patients with ampullary carcinoma were treated between 1965 and 1988: 22 underwent pancreaticoduodenectomy with 1 operative death (5 percent), 1 had local excision, 3 had bypass, and 2 were not explored. Of the 21 patients who survived pancreaticoduodenectomy, 4 had tumor confined to the ampulla, 7 had tumor extending into the duodenum, and 10 had tumor invasion beyond the duodenum. Nine of these patients had positive lymph nodes and 12 had negative lymph nodes. The patient who had local excision was disease-free at last follow-up 104 months postoperatively. Each of the three bypassed patients died of tumor progression within 15 months. The estimated 5-year survival rate for resected patients was 60 percent and was independently related to lymph node metastases (p = 0.031) and to tumor size (p = 0.039). This experience suggests that long-term survival is possible in patients with lymph node metastases or invasive tumors extending beyond the duodenal wall and that curative pancreaticoduodenectomy can be performed with a low operative mortality; therefore, aggressive surgical resection is recommended for all patients with ampullary carcinoma.     

A case of familial adenomatous polyposis complicated by thyroid carcinoma, carcinoma of the ampulla of vater and adrenocortical adenoma.

A 63 year old female with familial adenomatous polyposis (FAP) complicated by carcinoma of the thyroid and ampulla of Vater and a nonfunctioning adrenocortical adenoma is reported herein. We reviewed 23 cases of duodenal or periampullary carcinoma, 10 cases of thyroid carcinoma and 4 cases of adrenal neoplasm presented in the Japanese literature. From studying these cases, we identified that FAP patients had a high risk of developing periampullary carcinoma and consequently, it is very important to periodically examine the ampulla of Vater. Thyroid carcinomas in patients with FAP were well differentiated carcinomas and occurred earlier than in the general population. The use of medical imaging equipment with mean that insidious abdominal tumors in cases of FAP will be detected more frequently in the future and as patients with FAP have a high risk of developing malignancies, surgery should be indicated for incidentally found adrenal tumors.     

Clinical behavior and prognostic factors of periampullary adenocarcinoma.

OBJECTIVE: The authors evaluated the outcome and potential prognostic factors of 60 patients with surgically resected periampullary tumors. SUMMARY BACKGROUND DATA: Periampullary carcinomas exhibit different clinical behaviors according to their site of origin. There are no prognostic factors for deciding the type of surgery to be used or for choosing patients with tumors that have a poor prognosis for adjuvant treatment. METHODS: A retrospective review was performed of 15 clinical and pathologic variables encountered among 60 patients with periampullary tumors. Tumors were divided into four groups according to their site of origin. Kaplan-Meier survival curves of the four groups were plotted and differences were evaluated with the log-rank test. Cox's proportional hazards model was used to test for separate and combined independent predictors of disease-free survival. RESULTS: Twenty-nine ampullary carcinomas, 20 ductal pancreatic carcinomas, 7 distal common bile duct carcinomas, and 4 carcinomas of the periampullary duodenum were found. Five-year disease-free survival was 43%, 0%, 0%, and 75%, respectively. According to the Cox analysis, absence of neural invasion and use of adjuvant chemotherapy were significant factors for longer survival of patients with ampullary tumors. Lymphatic invasion was related to a shorter survival in patients with pancreatic carcinoma. CONCLUSIONS: Five-year disease-free survival of patients with periampullary tumors is related to tumor type. Prognosis was better for ampullary tumors if neural invasion was absent and if adjuvant chemotherapy was used. Lymphatic invasion was associated with a shorter recurrence-free survival among patients with pancreatic carcinoma.     

A case of familial adenomatous polyposis complicated by thyroid carcinoma,carcinoma of the ampulla of vater and adrenocortical adenoma

A 63 year old female with familial adenomatous polyposis (FAP) complicated by carcinoma of the thyroid and ampulla of Vater and a nonfunctioning adrenocortical adenoma is reported herein. We reviewed 23 cases of duodenal or periampullary carcinoma, 10 cases of thyroid carcinoma and 4 cases of adrenal neoplasm presented in the Japanese literature. From studying these cases, we identified that FAP patients had a high risk of developing periampullary carcinoma and consequently, it is very important to periodically examine the ampulla of Vater. Thyroid carcinomas in patients with FAP were well differentiated carcinomas and occurred earlier than in the general population. The use of medical imaging equipment with mean that insidious abdominal tumors in cases of FAP will be detected more frequently in the future and as patients with FAP have a high risk of developing malignancies, surgery should be indicated for incidentally found adrenal tumors.     

Intestinal-type and Pancreatobiliary-type Adenocarcinomas: How Does Ampullary Carcinoma Differ from Other Periampullary Malignancies?

Background

Ampullary carcinomas typically have either intestinal or pancreatobiliary type of differentiation, histopathologically resembling carcinomas of its adjacent tissues (duodenum, bile duct, or pancreas). We evaluated whether the histologic type itself is more important for long-term survival than the fact that the tumor originated in the ampulla.

Methods

Microscopic slides from 207 consecutive pancreatoduodenectomies were reviewed (72 pancreatic, 46 biliary, 61 ampullary, and 28 duodenal adenocarcinomas; 76 intestinal type, 131 pancreatobiliary type). Tumor size, nodal involvement, margin involvement, degree of differentiation, vascular involvement, and perineural growth, as well as overall survival, were compared between different origins of the same histologic type.

Results

Intestinal-type ampullary adenocarcinomas had similar frequency of poor histopathologic factors compared to duodenal adenocarcinomas, and pancreatobiliary-type ampullary adenocarcinomas had similar frequency of poor histopathologic factors compared to pancreatobiliary-type biliary and pancreatic adenocarcinomas. Adjusting for tumor size and nodal involvement, there was no difference in long-term survival between patients with intestinal-type ampullary, duodenal, or biliary and pancreatic tumors (p = 0.79), and there was no difference in long-term survival between patients with pancreatobiliary-type ampullary, biliary, or pancreatic tumors (p = 0.41).

Conclusions

Long-term survival for patients with ampullary carcinomas equals pancreatic, biliary, and duodenal carcinomas when the same histologic type is compared. It can be questioned whether ampullary carcinomas should be regarded as a separate entity in classification of solid tumors. Clinical trials on adjuvant treatments for periampullary carcinomas should stratify by pancreatobiliary type versus intestinal type of histologic differentiation.     

Telomerase activity in periampullary tumors correlates with aggressive malignancy

OBJECTIVE: To determine the presence of telomerase activity in a variety of periampullary malignancies and pancreatic diseases and quantify its activity to establish any association with the stage or aggressiveness of malignancy. SUMMARY BACKGROUND DATA: Progressive shortening of telomeres, repetitive DNA sequences at the ends of chromosomes, plays a role in cell senescence. Telomerase catalyzes conservation of telomeric repeats and may promote cell immortality and hence malignancy. It is absent in normal tissues but upregulated in more than 80% of cancers. METHODS: Fresh specimens of 62 periampullary tumors were snap-frozen in liquid nitrogen and adjacent tissue was formalin-fixed for histopathology. The telomerase repeat amplification protocol (TRAP) was used to obtain telomerase DNA products. These were separated with gel electrophoresis, stained with SYBR green, and quantified by densitometry. Findings were confirmed with a fluorometric TRAP assay in which fluorescent primers specific for telomerase were selectively amplified in its presence. RESULTS: Telomerase activity was upregulated in 26 of 33 periampullary malignancies (79%): 17 of 21 pancreatic adenocarcinomas (81%), 2 of 2 cholangiocarcinomas, 2 of 2 duodenal carcinomas, and 5 of 8 ampullary carcinomas (63%). Poorly differentiated periampullary tumors had significantly higher telomerase activity than well-differentiated tumors, and tumors larger than 2 cm had significantly higher telomerase activity than those 2 cm or smaller. Pancreatic ductal adenocarcinomas with lymph node metastases had significantly greater activity than node-negative cancers. Two of 11 intraductal papillary mucinous tumors were positive for telomerase activity, but only in foci of invasive carcinoma. Chronic pancreatitis (n = 7), serous cystadenomas (n = 5), benign mucinous cystic neoplasms (n = 4), neuroendocrine cancer (n = 1), and acinar cell carcinoma (n = 1) had no detectable telomerase activity. CONCLUSION: Telomerase activity is common in periampullary carcinomas. The magnitude of activity correlates with aggressiveness in pancreatic adenocarcinoma and may prove useful as a molecular index for biologic staging.     

Transduodenal local resection for low-risk group ampulla of vater carcinoma

BACKGROUND: Carcinoma of the ampulla of Vater has a more favorable prognosis, compared to other malignant tumors of the periampullary region, because it usually presents with symptoms in the early stage. However, treatment by local resection only of the ampullary carcinoma remains controversial. The aim of this study was to evaluate the treatment results of the ampulla of Vater carcinoma according to different types of operation in low-risk-group patients. METHODS: We retrospectively reviewed the medical records of 17 low-risk-group patients among a total of 102 patients with ampulla of Vater carcinoma who had underwent curative surgery from 1992 to 2002. All specimens were critically reviewed by a single expert pathologist, and the relationship between surgical outcomes and operation type was assessed. RESULTS: The low-risk group was comprised of 10 men and 7 women with a median age of 57.8 years. Thirteen of 17 patients underwent a pancreaticoduodenectomy (PD) or a pylorus preserving pancreaticoduodenectomy (PPPD), while 4 patients underwent a transduodenal local resection (TDLR). The operation time was significantly shorter in the TDLR group, compared to the PD or PPPD groups. Among the 17 patients, there was only 1 case of recurrence in the inguinal area 33 months after the pancreaticoduodenectomy. CONCLUSIONS: Transduodenal local resection is a comparable mode of operation for low-risk-group patients with Ampulla of Vater carcinoma. In particular, it is essential to evaluate the invasion depth in preoperative endoscopic ultrasonography, cell differentiation in preoperative biopsy, and positivity of resection margin accurately by using frozen section during the operation.     

影像学检查在壶腹周围癌诊断中的应用价值

目的 探讨各种影像学检查在壶腹周围癌诊断中的应用价值。方法 对我院1991年1月至2000年12月10年间收治的125例经病理组织学检查证实的壶腹周围癌患者的临床影像学检查资料进行回顾性分析。结果 胰头癌中BUS或CT的诊断阳性发现率高于ERCP(P=0．044，P=0．029)；十二指肠乳头癌中ERCP的诊断阳性发现率高于BUS和CT(P=0．005，P=0．03)；壶腹癌中ERCP的诊断阳性发现率高于BUS和CT(P=0．157，P=0．282)。磁共振胰胆管成像(MRCP)在壶腹癌和十二指肠乳头癌的诊断中阳性发现者分别为8／8和8／9。结论 BUS CT检查是诊断胰头癌的最佳方法；ERCP是诊断壶腹癌和十二指肠乳头癌的最佳方法；而MRCP检查应用前景十分广阔。     

Experience with 647 consecutive tumors of the duodenum, ampulla, head of the pancreas, and distal common bile duct.

Between 1946 and 1987, 647 patients with periampullary tumors were diagnosed at the University of Chicago Medical Center. These included 549 tumors located in the head of the pancreas, 40 in the distal common bile duct, 29 in the duodenum, and 29 at the ampulla of Vater. Ninety-eight per cent of all tumors were adenocarcinoma, with 93% of the remaining being duodenal carcinoid or sarcoma. Operability rate ranged from 81% to 97%, according to the tumor location and histologic type. A combination of laparotomy, biopsy, and bypass was performed in 433 patients and only one survived 5 years (0.2%). Resectability rate ranged from 16.5% for pancreatic adenocarcinoma to 89.3% for ampullary tumors. Of the 133 resections, 80 were pancreatoduodenectomies, 29 total pancreatectomies, 7 duodenectomies, 2 gastrectomies, 8 common bile duct resections, and 7 local excisions. Overall 19% of patients who underwent radical resection died in the immediate postoperative period, although mortality has decreased to 5% since 1981. Mortality was 20% after a standard pancreatoduodenectomy and 24.1% after a total pancreatectomy. Five-year actuarial survival rates, including perioperative deaths, were 8.8%, 20%, and 32% for pancreatic, duodenal, and ampullary adenocarcinoma, respectively. One half of patients with sarcoma and two-thirds with carcinoid of the duodenum survived 5 years. No patient with distal common bile duct adenocarcinoma achieved a 5-year survival rate. Multivariate analysis on all patients operated on (n = 566) revealed that the 5-year survival rate was significantly related to intent of operation (palliative 0.2%, curative 12%; p less than 0.001), histologic type (adenocarcinoma 2%, carcinoid and sarcoma 31%; p less than 0.0001), and site (ampullary and duodenal 21%, biliary and pancreatic 0.9%; p less than 0.001). A second multivariate analysis, evaluating only those patients with adenocarcinoma who survived the perioperative period of the radical resection (n = 97) analyzed the influence of tumor size and differentiation, lymphatic, capillary, and perineural microinvasion, lymph node status, and type of procedure (pancreatoduodenectomy vs. total pancreatectomy) on 5-year survival. None of these additional variables was significantly associated with long-term survival rates. In addition we evaluated the presence of local or distant recurrence after resection by analyzing the findings from all autopsies performed on these patients (n = 49): 29.4% of patients died with local recurrence alone, 23.5% with distant recurrence alone, and 47.1% had both local and distant recurrences.(ABSTRACT TRUNCATED AT 400 WORDS)     

Periampullary adenocarcinoma: analysis of 5-year survivors.

OBJECTIVE: This single-institution experience retrospectively reviews the outcomes in a group of patients treated 5 or more years ago by pancreaticoduodenectomy for periampullary adenocarcinoma. SUMMARY BACKGROUND DATA: Controversy exists regarding the benefit of resection for periampullary adenocarcinoma, particularly for pancreatic tumors. Many series report only Kaplan-Meier actuarial 5-year survival rates. There are believed to be discrepancies between the actuarial 5-year survival data and the actual 5-year survival rates. METHODS: From April 1970 through May 1992, 242 patients underwent pancreaticoduodenal resection for periampullary adenocarcinoma at The Johns Hopkins Hospital. Follow-up was complete through May 1997. All pathology specimens were reviewed and categorized. Actual 5-year survival rates were calculated. The demographic, intraoperative, pathologic, and postoperative features of patients surviving > or =5 years were compared with those of patients who survived <5 years. RESULTS: Of the 242 patients with resected periampullary adenocarcinoma, 149 (62%) were pancreatic primaries, 46 (19%) arose in the ampulla, 30 (12%) were distal bile duct cancers, and 17 (7%) were duodenal cancers. There was a 5.3% operative mortality rate during the 22 years of the review, with a 2% operative mortality rate in the last 100 patients. There were 58 5-year survivors, 28 7-year survivors, and 7 10-year survivors. The tumor-specific 5-year actual survival rates were pancreatic 15%, ampullary 39%, distal bile duct 27%, and duodenal 59%. When compared with patients who did not survive 5 years, the 5-year survivors had a significantly higher percentage of well-differentiated tumors (14% vs. 4%; p = 0.02) and higher incidences of negative resection margins (98% vs. 73%, p < 0.0001) and negative nodal status (62% vs. 31%, p < 0.0001). The tumor-specific 10-year actuarial survival rates were pancreatic 5%, ampullary 25%, distal bile duct 21%, and duodenal 59%. CONCLUSIONS: Among patients with periampullary adenocarcinoma treated by pancreaticoduodenectomy, those with duodenal adenocarcinoma are most likely to survive long term. Five-year survival is less likely for patients with ampullary, distal bile duct, and pancreatic primaries, in declining order. Resection margin status, resected lymph node status, and degree of tumor differentiation also significantly influence long-term outcome. Particularly for patients with pancreatic adenocarcinoma, 5-year survival is not equated with cure, because many patients die of recurrent disease >5 years after resection.     

Pancreaticoduodenectomy. A 40-year experience.

One hundred six pancreaticoduodenectomies, including 10 cases done for benign disease were reviewed, retrospectively, and 51 factors analyzed. There is a significant difference in survival based upon final pathologic diagnosis. Five-year survivals were 0% for adenocarcinoma of the pancreas, 38% for duodenal carcinoma, 24% for CBD caracinoma, 30% for ampullary carcinoma, 24% for other cancers not considered part of the ampullary carcinoma, 24% for other cancers not considered part of the ampullary region, and 100% for those with benign disease (chi 2 = 24.66, df = 5, p = 0.0002). Four statistically significant poor prognostic factors were identified, including age greater than 51 years, serum bilirubin level greater than 6 mg/dl, SGOT greater than 100, and a previous drainage procedure. Results of this study seem to indicate that the pancreaticoduodenectomy has limited indications in adenocarcinoma of the pancreas, but remains the procedure of choice for all other periampullary lesions including suspicious lesions with a negative biopsy at the time of surgery.     

Carcinoma of the Ampulla of Vater Associated with Annular Pancreas

Carcinoma of the ampulla of Vater associated with annular pancreas is a rare entity. Only seven cases have been reported so far in the English literature. We herein report one such case in a 42-year-lady who presented with progressively increasing jaundice and pain in the upper abdomen for past 2 months. A magnetic resonance cholangiopancreaticography (MRCP) and MRI revealed an annular pancreas with an ampullary mass and a dilated CBD. An upper gastrointestinal endoscopic biopsy from the ampullary mass revealed a moderately differentiated adenocarcinoma. She was diagnosed as having annular pancreas with periampullary cancer that was subsequently confirmed at laparotomy. Histological examination confirmed a complete annular pancreas with a 2 cm × 2 cm moderately differentiated adenocarcinoma of the ampulla of Vater. The management, however, remains as in any case of periampullary malignancy. This highlights the importance that obstructive jaundice in an adult patient presenting with annular pancreas may be associated with a coexisting periampullary malignancy.