The management of tumors of the ampulla of Vater by local resection.

OBJECTIVE: The authors report on indications and results of local excision of tumors of the ampulla of Vater. SUMMARY BACKGROUND DATA: Local excision of ampullary tumors has been performed for nearly a century but remains controversial. The use of this procedure for benign conditions is clear, but its place, if any, in the management of ampullary malignancy is debated. METHODS: The presentation, evaluation, and treatment of 26 patients who underwent local resection of ampullary tumors between January 1987 and November 1994 are reviewed. RESULTS: There were 16 men and 10 women, with a median age of 58 years. Eighteen patients had adenomas, whereas 8 patients had adenocarcinomas. Patients presented predominantly with jaundice (50%), pain (35%), and pancreatitis (27%) and were evaluated with endoscopic retrograde cholangiopancreatography and biopsy. All patients with benign lesions had accurate preoperative biopsies. Two of eight patients shown intraoperatively to have malignant lesions had preoperative biopsies read as benign. There were no deaths. Postoperative complications included two wound infections and one episode each of cholangitis, lower gastrointestinal bleeding, and adhesive gastrointestinal obstruction. All patients had prompt resolution of jaundice if present before surgery, and the mean postoperative stay was 7.5 days. Six of eight patients with malignant lesions have had recurrent disease. CONCLUSIONS: Local excision of malignant ampullary tumors is effective palliative therapy when the patient is unfit for the Whipple procedure. Ampullary resection usually is curative for patients with benign lesions without a polyposis syndrome. In this series, intraoperative frozen section routinely was accurate.     

Ampullary tumors: endoscopic versus operative management

Most ampullary adenomas (80%) are common benign ampullary tumors; however, they can range from mild dysplasia to high-grade dysplasia to invasive carcinoma. They are considered premalignant lesions found in the setting of familial polyposis syndromes or found sporadically, usually manifested by vague abdominal pain, liver enzyme elevation, jaundice, recurrent pancreatitis, or with uncommon symptoms such as gastrointestinal bleeding or duodenal obstruction. Endoscopic retrograde cholangiopancreatography with biopsy is a minimally invasive technique used to visualize these tumors directly and to evaluate their histologic characteristics. Definitive treatment primarily depends on these histologic results. Local resection has a high rate of recurrence (5% to 30%) and requires postoperative endoscopic surveillance, which is the reason it is not considered as a first choice in the management of ampullary tumors. The operative mortality is 10% or less for pancreaticoduodenectomy, a procedure of choice at most experienced centers for frank carcinoma, foci papillary adenocarcinoma in pre-excisional biopsies, or high-grade dysplasia ampullary adenomas. Endoscopic interventions for presumed benign ampullary adenomas have resolved symptoms of obstruction, but long-term follow up is necessary to detect early malignant transformation. In summary, the choice of treatment depends on level of surgical skill available, patient tolerance of long-term endoscopic surveillance versus radical surgery, and the presence or absence of coexisting familial adenomatous polyposis.     

Safety and long-term efficacy of transduodenal excision for tumors of the ampulla of Vater

BACKGROUND: Ampullary tumors should be resected because of the high incidence of malignancy and the unreliability of preoperative endoscopic diagnosis. Controversy exists about whether to perform a transduodenal excision (TDE) or a pancreatoduodenectomy. This study evaluated the safety and long-term efficacy of TDE. METHODS: The records of 21 patients with a pathologic diagnosis of ampullary adenoma who underwent TDE were reviewed. Demographics, symptoms, pathologic findings, and outcomes were analyzed and long-term follow-up was ascertained. RESULTS: Twenty-one patients (mean age, 61 years) underwent TDE. Final pathology showed adenoma in all patients including 1 (5%) with invasive cancer, 2 (9%) with microinvasive cancer, 6 (28%) with high-grade dysplasia, and 1 (5%) with low-grade dysplasia. The overall survival was 85% (mean follow-up of 38 months). One of 3 late deaths was likely related to disease progression. Sixteen of the 18 remaining patients (89%) had no evidence of tumor recurrence. One benign ampullary recurrence was successfully treated endoscopically. One additional patient developed an ampullary cancer and underwent pancreatoduodenectomy. CONCLUSIONS: TDE of benign ampullary tumors, even those with varying grades of dysplasia, can be performed with acceptable morbidity and low rates of recurrence. Postoperative endoscopic surveillance is mandatory to identify recurrent tumors.     

Treatment of ampullary villous adenomas that may harbor carcinoma

Villous adenoma of the ampulla of Vater is a rare tumor. It is a deceptive tumor because it is a premalignant lesion and biopsies of the lesion are false negative in 25% to 56% of patients. The primary focus of this report is 23 of 30 patients with villous adenoma of the ampulla of Vater who underwent Whipple operations. Paraffin blocks from 22 patients were available. In eight patients, blocks of the biopsies and the corresponding resected specimens were available. Immunohistochemical studies using antibodies to p53 and Ki-67 were performed to determine whether accumulation of these antibodies in the biopsy specimens would identify false negative biopsies. There was one operative death. The 2-, 5-, and 10-year survival rates for the 22 patients surviving a Whipple operation were 74%, 57%, and 35%, respectively. Three patients died of cancer. The mean p53 expression index was increased in adenomas to 88 (P = 0.001) and in carcinomas to 114 (P = 0.01), compared with 12.6 for normal ampullary epithelium adjacent to tumor. Significant differences in the Ki-67 proliferation index were noted between normal adjacent epithelium (13%), adenoma (34%, P = 0.0002), and carcinoma (53%, P = 0.034), as well as between adenomatous epithelium and carcinoma (34% vs. 53%, P = .012). Villous ampullary adenocarcinoma was present in 65% of patients with villous adenoma (87% if patients with carcinoma in situ in resected specimens are included). Because of the high false negative rate of ampullary biopsies, and the inability to accurately stage these lesions, we recommend pancreaticoduodenectomy in most patients. Studies with p53 and Ki-67 markers suggest that they may be helpful in the recognition of ampullary villous cancer not identified on routine biopsies.     

Local ampullary resection with careful intraoperative frozen section evaluation for presumed benign ampullary neoplasms

BACKGROUND: Frozen section evaluation has been reported to be inaccurate in detecting foci of adenocarcinoma within adenomas of the ampulla of Vater, leading many authors to advocate pancreaticoduodenectomy as the method of treatment for these neoplasms. The authors hypothesized that (1) ampullary resection is less morbid than pancreaticoduodenectomy, and (2) frozen section evaluation following ampullary resection is accurate and allows for a selective application of pancreaticoduodenectomy to those with carcinoma or benign lesions too large to be locally resected. METHODS: A retrospective review of a single-surgeon experience was conducted. Thirty-eight patients who underwent ampullary resection and pancreaticoduodenectomy (39 procedures) for benign and malignant ampullary neoplasms were identified. Our technique of step-frozen section analysis is described. RESULTS: Twenty-one ampullary resections were performed for preoperative diagnoses of benign (16) and malignant (5) ampullary neoplasms. Frozen section evaluation accurately predicted the final histology in all patients undergoing ampullary resection. Ampullary resection (vs pancreaticoduodenectomy) was associated with a statistically lower operative time (169 minutes vs 268 minutes), estimated blood loss (192 mL vs 727 mL), mean length of stay (10 days vs 25 days), and overall morbidity (29% vs 78%). CONCLUSIONS: Frozen section evaluation of ampullary neoplasms is accurate. Because ampullary resection is less morbid than pancreaticoduodenectomy and frozen section evaluation is accurate, ampullary resection with frozen section evaluation is our current approach to the treatment of small benign ampullary neoplasms.     

Duodenal villous tumors

The treatment of 36 patients with duodenal villous tumors was reviewed to determine the long-term outcome of various surgical treatment options for specific adenoma histopathology. Duodenal villous tumors were typically solitary and periampullary in location. Villous adenomas contained epithelial atypia in 30% of patients, in situ carcinoma in 14%, and invasive carcinoma in 33%. Treatment consisted of transduodenal submucosal excision in 19 patients and radical pancreaticoduodenectomy in 15. There was no perioperative mortality. Perioperative morbidity for transduodenal excision and pancreaticoduodenectomy was 16% and 47%, respectively. Benign adenomas recurred more than 5 years postoperatively in 17% of patients undergoing transduodenal excision. Five-year survival following radical resection for invasive cancers was 45%. Overall median follow-up was 5.8 years. We conclude that duodenal villous tumors without invasive cancer can be managed successfully by local submucosal excision, but invasive carcinoma requires radical resection.     

Impact of grade of dysplasia in villous adenomas of Vater's papilla

Therapeutic strategies for villous adenoma of the papilla of Vater remain controversial. This study evaluates the accuracy of preoperative histopathologic diagnosis and the impact of the grade of dysplasia on recurrence as well as on potential alteration of the surgical approach. A series of 32 patients with an adenoma of Vater's papilla who underwent local resection or pylorus-preserving pancreaticoduodenectomy between January 1990 and August 2000 were reviewed retrospectively. Multiple endoscopic biopsies had been performed preoperatively. The histopathology of the preoperatively obtained biopsy specimens and subsequent surgical specimens were evaluated for grade of dysplasia by two pathologists and correlated with the clinical course after operative treatment. Altogether, 3 of 11 patients (27%) with a low-grade (LG) dysplasia adenoma and 6 of 21 patients (29%) with a high-grade (HG) dysplasia adenoma in the initial endoscopic biopsy specimens exhibited invasive carcinoma at the postoperative histologic examination (NS). Recurrence was not observed in the 6 patients from the LG dysplasia adenoma group following local resection and benign postoperative histology. In contrast, recurrence of villous adenoma was discovered in 2 of 12 patients (17%) and development of invasive carcinoma in 5 of 12 patients (42%) from the preoperative HG dysplasia group (p <0.05). The overall risk of carcinoma after primary diagnosis of an HG dysplasia adenoma was 44% (14/32). Adenoma of the papilla of Vater including HG dysplasia appears to be associated with a high risk of exhibiting invasive carcinoma postoperatively and a high rate of recurrence. Therefore pylorus-preserving pancreaticoduodenectomy should be offered to patients with an HG dysplasia adenoma.     

Safety and long-term follow-up of endoscopic snare excision of ampullary adenomas

Background Adenomas of the duodenal papilla are rare. Because of their malignant potential, resection is mandatory. Options for resection include endoscopic resection techniques, transduodenal local excision, and pancreaticoduodenectomy. The aim of this retrospective study was to evaluate the safety and outcome of endoscopic snare resection of papillary adenomas in a Greek cohort of patients. Methods Fourteen patients (six women and eight men; age range, 42–76 years) were referred for endoscopic management of ampullary adenomas. A questionnaire was completed for each patient, which included preoperative and postoperative data points. Presenting symptoms were jaundice (n = 4), cholangitis (n = 1), and pain (n = 2). Seven patients were asymptomatic. If there was no common bile and main pancreatic duct invasion and the appearance suggested a benign lesion, biductal sphincterotomy onto normal duodenal tissue was performed. The adenomas were resected via a diathermy snare, along with the major papilla, after elevation of the lesion by epinephrine plus dextrose 50% (1:10,000) solution. At the discretion of the endoscopist, a biliary or pancreatic stent was inserted as a prophylactic procedure immediately after excision. Results Histopathologically, resected tissue included 11 adenomas and three adenomas with focal malignancy, referred for pancreaticoduodenectomy. Immediate complications were moderate bleeding (n = 1) and mild pancreatitis (n = 1). No procedure-related death occurred. Follow-up was available for 11 patients (mean, 28.36 months; range, 6–72). Pancreatic and biliary stents were placed in four and nine patients, respectively. Follow-up endoscopy revealed recurrent/residual adenomatous tissue in two patients (18%), which was resected endoscopically. Conclusion Endoscopic snare resection of adenomas of the major duodenal papilla is a safe, well-tolerated alternative to surgical therapy. In expert hands, complications are mild and may be avoided by pre-resection biductal sphincterotomy, stent placement, and elevation of the lesion by epinephrine plus dextrose 50% solution injection.     

Local resection of ampullary adenocarcinomas of the duodenum

Background : Pancreaticoduodenectomy (PD) is considered to be the optimal treatment for ampullary adenocarcinomas. Local resection (LR) is a less invasive and potentially equally effective alternative for cancers with favourable prognostic features. Identification of these prognostic parameters may allow selection of patients suitable for LR. Methods : Twenty‐five patients were treated for a primary Vater’s ampulla adenocarcinoma at the Alfred Hospital, Melbourne, Australia, between January 1989 and January 2000. Risk factors for cancer recurrence were evaluated and the specific role of LR was defined. Results : Fourteen patients had PD, five had LR and six had bypass procedures (five biliary stents; one operative bypass). Presenting symptoms included jaundice (64%), abdominal pain (54%) and weight loss (32%). Adenocarcinomas that were resected had a median diameter of 2.5 cm, and were limited to the ampulla in 26% (T₁), invaded the duodenal wall in 42% (T₂) and infiltrated 2 cm or less into the pancreas in 32% (T₃) of cases. Locally resected cancers were confined to the ampulla or invaded the duodenum and recurred in one patient following excision. Six recurrences occurred in total, influenced significantly by T staging (P = 0.009). Patient age, preoperative symptoms, laboratory tests, tumour size, differentiation, ulceration, lymphovascular spread and perineural invasion had no effect on recurrence. Patients undergoing LR had lower morbidity and mortality, reduced blood transfusion requirements and shorter hospital stay than those treated by PD. Conclusions : T staging predicts the risk of tumour recurrence and can be determined using endoscopic ultrasound. Local resection is a suitable alternative to pancreaticoduodenal resection in patients with T₁ and T₂ adenocarcinomas with a maximum diameter of 3 cm or less.     

Endoscopic resection of ampullary tumors: 12-year review of 21 cases

Background  Endoscopic snare papillectomy is increasingly performed with curative intent for benign papillary tumors. This study aimed to evaluate the outcome of endoscopic resection for ampullary tumors at a single center. Methods  All ampullary tumors without macroscopic features of malignancy identified by the endoscopic retrograde cholangiopancreatography (ERCP) from January 1995 to February 2007 were included in the study. Papillectomy was performed by snare resection using electrocautery. Argon plasma coagulation was effective for fulguration of small tissue remnants not amenable to snare resection. Results  Of the 21 patients (9 men and 12 women; mean age, 67.2 ± 14.3 years) evaluated, 11 had adenoma (7 had low-grade dysplasia [LGD] and 4 had high-grade dysplasia [HGD]), and 10 had carcinoma. All the patients underwent papillectomy. Of the 21 patients, 18 had extraductal growth or minimal intraductal growth, and 3 had extensive intraductal growth. The endoscopic complications (23.8%) included one case of mild bleeding, two cases of mild pancreatitis, and two cases of moderate pancreatitis. After papillectomy, 15 patients underwent Whipple procedures (endoscopic failure, 74.1%), including 3 patients with extensive intraductal growth (complete removal of the lesion impossible), 9 patients with carcinoma beyond the mucosal layer, and 3 patients with recurrence treated surgically. Endoscopic success (28.5%) was obtained for the remaining six patients (4 with LGD and 2 with HGD). Papillectomy was determined to be curative after a mean follow-up period of 15.9 ± 14.9 months. Conclusions  In the hands of an experienced endoscopist, endoscopic papillectomy is a clinically effective treatment for ampullary tumors without invasive neoplasia. Evaluation of a prepapillectomy tumor extension is an important criterion for assessment of endoscopic success.     

Endoscopic characteristics of gastric adenomas suggesting carcinomatous transformation

Background  Currently, endoscopic resections, including endoscopic mucosal resection (EMR) and endoscopic submucosal dissection (ESD), are widely performed for the management of gastric neoplasia. This study aimed to evaluate the potential predictive factors for carcinomas on the basis of endoscopic features. Methods  This study investigated 114 samples from 114 patients. Gastric adenoma was diagnosed initially for all the patients. The endoscopic findings were reviewed for location, size, gross appearance, surface nodularity, ulceration, surface color, and number of biopsy samples. These variables were analyzed and compared between an adenoma group (51 cases) and a carcinoma group (63 cases) on the basis of postresection diagnosis. Results  The mean age of the patients was 62 years (range, 43–82 years), and 83 of the patients were men. The diameter of the lesions was 14.6 ± 8.2 mm in the adenoma group and 15.4 ± 7.4 mm in the carcinoma group. Depressed type, combined high-grade dysplasia, red discoloration, and mucosal ulceration were significant variables associated with carcinomas. In the multivariate analysis, combined high-grade dysplasia was a significant independent predictor of carcinomas. Conclusions  The results suggest that patients with high-grade dysplasia on forceps biopsies should be considered candidates for endoscopic resection. Characteristics of gastric adenomas such as a depressed type, red color, and ulceration that may have foci of carcinomas in other parts of the adenomas also should be considered for endoscopic resection.     

Local resection of tumors of the ampulla of Vater

Local resection of an ampullary tumor with reimplantation of the pancreatic and bile ducts was first described by William S. Halsted in 1899. Technical hazard and unsuitability in malignant ampullary tumors have unfortunately led to a disregard for this operation that is unwarranted. Radical pancreaticoduodenectomy is now the most common method of resecting benign and malignant ampullary tumors. Experience was gained with two high-risk patients with benign adenomatous polyps obstructing the ampulla of Vater. Their medical unsuitability for radical pancreaticoduodenectomy led us to revive the procedure of wide local excision of these tumors with reimplantation of the pancreatic and bile ducts. Operative time and blood loss were substantially less than radical resection and postoperative recoveries were relatively uncomplicated. Radical resection of benign ampullary tumors may be appropriate for good-risk patients in whom the risk of local recurrence outweighs the operative risk. We suggest that local resection of benign ampullary tumors is the procedure of choice in high-risk patients and that it be considered in palliation of limited local malignancies of the ampulla in high-risk patients.     

局部切除术治疗Vater壶腹肿瘤的临床研究

目的 探讨局部切除术治疗Vater壶腹肿瘤的疗效。方法对1994年2月牙2007年7月间的16例Vater壶腹肿瘤患者经局部切除术后的疗效进行回顾性分析。结果本组病例平均住院为13天,发生手术并发症1例,无围手术期死亡,术后1年、3年、5年存活率分别为93皇％、75％、37．5％。结论局部切除术治疗Vater壶腹肿瘤具有安全、疗效确切等优点．适合绝大部分的Vater壶腹肿瘤患者。     

Villous adenocarcinoma of the colon and rectum: a clinicopathologic study of 36 cases

Some colorectal adenocarcinomas show villous architecture with morphologic similarities to tubulovillous or villous adenomas. We reviewed 420 consecutive colorectal adenocarcinoma resection specimens and found that 95 tumors (23%) showed areas of villous architecture. Thirty-six tumors (8.6%) in 35 patients showed more than 50% villous architecture and were designated villous adenocarcinomas. Only 42% of the villous adenocarcinomas showed severe atypia and only 44% of the available pre-resection biopsies of these tumors were diagnosed as adenocarcinoma. Epithelial islands in desmoplastic stroma (EIDS) may be helpful in the diagnosis of these tumors. EIDS were found in 97% of the resection specimens for villous adenocarcinomas and none of 62 resection specimens for tubulovillous or villous adenomas. The presence of EIDS showed a 67% sensitivity, 100% specificity, and 100% predictive value in the diagnosis of villous adenocarcinoma in a blinded review of villous tumors. On review of the pre-resection biopsies of villous adenocarcinoma without a final diagnosis of adenocarcinoma, 40% showed EIDS. Clinical follow-up of the 35 patients with villous adenocarcinoma showed that only one died of colorectal adenocarcinoma (median follow-up, 46 months). This sole patient dying of colorectal adenocarcinoma showed a synchronous advanced stage of nonvillous adenocarcinoma at the time of diagnosis. Villous adenocarcinoma is a diagnostically challenging subset of colorectal adenocarcinoma, which appears to be associated with a favorable prognosis. Classifying these tumors as a special type of colorectal cancer may facilitate the development of diagnostic adjuncts and optimal treatment protocols.     

Villous tumors of the duodenum: Reappraisal of local vs. extended resection

Benign villous tumors of the duodenum are often managed by transduodenal local excision. Risk of local recurrence, coupled with improving safety of radical pancreaticoduodenectomy, has prompted reexamination of the roles of conservative and radical operations. The aim of this study was to determine long-term outcome after local and extended resection in order to identify factors to consider in planning operative strategy. Eighty-six patients (mean age 64 years) with villous tumors of the duodenum managed surgically from 1980 to 1997 were reviewed. Histologic findings, size, presence of polyposis syndromes, and extent of resection were correlated with outcome. Villous tumors were benign adenomas in 64 patients (74%), contained carcinoma in situ in three (4%), and invasive carcinoma in 19 (22%). The presence of cancer was not known preoperatively in 9 (47%) of the 19 with invasive carcinoma. Operative treatment included transduodenal local excision in 53 patients, pancreaticoduodenectomy in 20, pancreas-sparing duodenectomy in five, full-thickness excision in four, and other in six. Among the 50 patients with benign tumors managed by local excision, 17 had a recurrence with actuarial rates of 32% at 5 years and 43% at 10 years; four of the recurrences (24%) were adenocarcinomas. The recurrence rate was influenced by the presence of a polyposis syndrome but not by tumor size. Recurrence of benign villous tumors after local excision is common and may be malignant. Pancreaticoduodenectomy is appropriate for villous tumors containing cancer and may be considered an alternative for select patients with benign villous tumors of the duodenum. If local excision is performed, regular postoperative endoscopic surveillance is mandatory. Presented at the Fortieth Annual Meeting of The Society for Surgery of the Alimentary Tract, Orlando, Fla., May 16–19, 1999, and published as an abstract in Gastroenterology 116:A1310, 1999.     

Tumors of the ampulla of vater: histopathologic classification and predictors of survival

BACKGROUND: The histology and clinical behavior of ampullary tumors vary substantially. We speculated that this might reflect the presence of two kinds of ampullary adenocarcinoma: pancreaticobiliary and intestinal. STUDY DESIGN: We analyzed patient demographics, presentation, survival (mean followup 44 months), and tumor histology for 157 consecutive ampullary tumors resected from 1989 to 2006. Histologic features were reviewed by a pathologist blinded to clinical outcomes. Survival was compared using Kaplan-Meier/Cox proportional hazards analysis. RESULTS: There were 33 benign (32 adenomas and 1 paraganglioma) and 124 malignant (118 adenocarcinomas and 6 neuroendocrine) tumors. One hundred fifteen (73%) patients underwent a Whipple procedure, 32 (20%) a local resection, and 10 (7%) a palliative operation. For adenocarcinomas, survival in univariate models was affected by jaundice, histologic grade, lymphovascular, or perineural invasion, T stage, nodal metastasis, and pancreaticobiliary subtype (p < 0.05). Size of tumor did not predict survival, nor did cribriform/papillary features, dirty necrosis, apical mucin, or nuclear atypia. In multivariate models, lymphovascular invasion, perineural invasion, stage, and pancreaticobiliary subtype predicted survival (p < 0.05). Patients with pancreaticobiliary ampullary adenocarcinomas presented with jaundice more often than those with the intestinal kind (p = 0.01) and had worse survival. CONCLUSIONS: In addition to other factors, tumor type (intestinal versus pancreaticobiliary) had a major effect on survival in patients with ampullary adenocarcinoma. The current concept of ampullary adenocarcinoma as a unique entity, distinct from duodenal and pancreatic adenocarcinoma, might be wrong. Intestinal ampullary adenocarcinomas behaved like their duodenal counterparts, but pancreaticobiliary ones were more aggressive and behaved like pancreatic adenocarcinomas.     

Resection of benign duodenal neoplasms

Primary small bowel neoplasms (PSBN) are uncommon, accounting for less than 15 per cent of all gastrointestinal tumors. Benign duodenal neoplasms (BDN) are rare, comprising only 10 to 20 per cent of all PSBN. The treatment is generally surgical resection ranging from local excision to pancreaticoduodenectomy depending on size, location, and number of lesions. Patients undergoing surgical treatment for BDN at Vanderbilt University Medical Center from July 1984 to April 2006, were identified and reviewed retrospectively. Medical records were examined for demographics, operative details, results, and complications. Twenty-six patients were identified of which 56 per cent were male and the mean age was 56 +/- 14 years. Lesions were found throughout the duodenum, but the majority (62%) were ampullary. Nearly 75 per cent were adenomas, including over half with dysplasia. Operative interventions and complication rates were: duodenal resection with primary anastomosis (n=3, 0%), local excision (n=6, 50%), ampullary resection (n=10, 30%), and pancreaticoduodenectomy (n=7, 86%). There were no reoperations or mortalities. Mean followup was 14 months. BDN are an increasingly common problem in an era of frequent use of upper endoscopy. The surgical management of these lesions must be tailored to their size, number, location, and malignant potential. A wide variety of surgical procedures can be performed with acceptable morbidity.     

Survival After Resection of Ampullary Carcinoma: A National Population-Based Study

Background  Ampullary cancer is the second most common periampullary cancer, with a resection and survival rate more favorable than that for pancreatic cancer. However, most reports have been conducted at single institutions with small sample sizes, and results may not reflect the practices and outcomes in the community. Our objective was to complete a population-based analysis of patients undergoing resection for ampullary carcinoma and compare it with outcomes in the published literature. Methods  Patients diagnosed with ampullary cancer reported in the Surveillance, Epidemiology, and End Results program (1988–2003) were collected. Primary outcome was survival (5-year), and secondary outcome was stage at presentation. Comparisons were made with outcomes reported in the literature (resection rate, perioperative mortality, and 5-year survival). Results  Of the 3292 ampullary cancer patients, 1301 (40%) underwent resection. Thirty-seven percent presented with stage I tumors. Perioperative mortality (30 day) was 7.6% after resection, and 5-year survival was 36.8%. Few patients died if they survived at least 5 years. The cancer registry data showed less early stage disease, higher perioperative mortality, and lower 5-year survival compared with published reports. Conclusions  This is the largest population-based analysis of ampullary carcinoma. Resection rates and survival at the national level are lower, in general, compared with cancer center reports, which may have implications for regionalizing these procedures. Many patients surviving at least 5 years seem to be cured by surgical resection.     

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.     

Indications for and the technique of local resection of tumors of the papilla of Vater

Local resection of tumors of the papilla of Vater was performed in six cases (three benign adenomas and three adenocarcinomas). Pathology was suggested in all cases by endoscopic biopsy and confirmed by operative excision. A method of excision of the tumor and reconstruction of the pancreaticobiliary apparatus was used that eliminated all gross and microscopic disease. Only one of the patients with benign disease (familial polyposis) suffered from local recurrence. All three patients with malignant tumors developed local recurrences within 2 years of resection. Local resection seems to be a viable alternative to Whipple's resection in patients with benign tumors. In malignant cases, local resection should be considered only a palliative procedure and confined to elderly patients with severe concomitant disease and patients in whom a more radical procedure would incur an undue surgical risk.