Collision Tumour of the Ampulla of Vater: Carcinoid and Adenocarcinoma

Obstructive jaundice is most commonly due to luminal stones or lesions of the head of the pancreas and more rarely ampullary and primary common bile duct lesions. Obstruction due to lesions of the ampulla of Vater may be due to adenocarcinoma which has a significantly better long term prognosis than carcinomas located in the head of the pancreas. A case is presented where two tumours were identified at the ampulla of Vater of the resected specimen one an adenocarcinoma and the other a carcinoid tumour representing a collision tumour.     

Patterns and Predictors of Failure after Curative Resections of Carcinoma of the Ampulla of Vater

Background:Curative resection does not always equate with long-term survival. The aim was to identify patterns and predictors of failure and independent factors of prognosis after curative resection.Methods:Sixty-six patients with ampullary carcinoma who underwent surgical intervention were reviewed. Fifty-nine patients underwent pancreaticoduodenectomy. Cox regression analysis, log-rank test, Fisher exact test, or ² test was used.Results:No patient died as a result of surgery; major complications occurred in three, and the 5-year survival rate after curative resection (n = 55) was 52.6%. Significant survival predictors were preoperative serum carcinoembryonic antigen level; gross tumor appearance; tumor, node, and tumor node metastasis stage; and microscopic lymphatic vessel and venous invasion in the primary tumor. Multivariate analysis demonstrated that lymphatic vessel invasion, tumor, and tumor node metastasis stage were significant independent prognostic factors. No patient experienced locoregional failure alone; all 24 relapsed patients had distant failure, and six of them had both. The liver was the most frequent metastatic organ, followed by nodes, peritoneum, lung, and bone. The carcinoembryonic antigen and carbohydrate antigen levels and lymphatic vessel and venous invasion were significant predictors of distant failure, and the mean time to relapse was 13 (range, 0.7–33) months.Conclusions:Curative resection is associated with significant survival; however, effective systemic adjuvant therapy is needed to prevent distant failure for patients with elevated carcinoembryonic antigen and carbohydrate antigen levels or positive lymphatic vessel or venous invasion. A 3-year follow-up period would be necessary to document relapses.     

Defining the Role of Adjuvant External Beam Radiotherapy on Resected Adenocarcinoma of the Ampulla of Vater

The role of adjuvant radiotherapy in the treatment of ampullary carcinoma (AC) remains unclear. We hypothesized that adjuvant radiotherapy (RT) does not improve survival following resection for AC. The SEER database was queried for patients with non-metastatic AC who underwent surgery (S) from 2004 to 2010. Propensity score (PS) modeling was applied to create balanced cohorts of patients that would be equally likely to receive RT. Cox proportional hazard models were used to compare survival. Of 1,287 patients, 329 (25.6 %) received adjuvant RT. Unadjusted median overall survival (OS) for patients receiving adjuvant RT compared to S alone was 27 vs. 36 months (p?=?0.14). Patients receiving RT were younger (63 vs. 69 years, p?p?p?p?=?0.58) and disease-specific survival (36 vs. 40 months, p?=?0.92) in propensity-matched cohorts, although certain imbalances remained between treatment groups. Adjuvant RT does not confer a survival benefit for patients with ampullary tumors. The lack of disease-specific survival benefit suggests that it may also not be beneficial to prevent local recurrences.     

Villous Adenocarcinoma of the Duodenum Invading the Ampulla of Vater: Case Report and Review of Literature

We report a case of villous adenocarcinoma of duodenum arising from the ampulla of Vater with a review of the literature. Although preoperative endoscopic biopsies were performed, no malignancy was identified. Because of the pathological uncertainty we decided to perform a pylorus-preserving pancreatoduodenectomy. Microscopic examination demonstrated glandular dysplasia with aspects of villous adenoma and well differentiated adenocarcinoma. We conclude that both in malignant cases and in cases with uncertain diagnosis a pylorus-preserving pancreatoduodenectomy is the best surgical treatment because it results in better 5 year survival.     

Vater壶腹结石嵌顿的术中处理

目的探讨Vater壶腹结石嵌顿的术中处理技巧。方法回顾性分析2003年8月~2006年12月共35例此类病人的临床资料。30例经胆总管下段切开取石,2例行经十二指肠Odd i括约肌切开取石,3例行胆道镜下微爆破碎石仪碎石术。结果所有手术均成功,无一例病人出现大出血、胆漏、肠漏等严重的并发症。平均随访18个月,均无腹痛及胆总管结石复发。结论Vater壶腹嵌顿结石的处理应根据病人病情及医疗条件抉择,在剖腹术中采取经胆总管下段切开取石较为简便和安全。     

Traumatic Duodenal Rupture and Avulsion of the Ampulla of Vater

Duodenal injury following blunt abdominal trauma is uncommon. The severity of injury can vary from an intramural haematoma to a duodenal rupture with associated transection of the pancreatic duct. A case of duodenal rupture with avulsion of the ampulla of Vater is presented and discussed.     

Treatment Options for Villous Adenoma of the Ampulla of Vater

Introduction: Duodenal villous adenoma arising from the ampulla of Vater has a high risk of malignant development. Excluding associated malignant disease prior to resection of an adenoma of the ampulla is not always possible. Therefore, the surgical procedure of choice to treat this rare tumour is still controversial.Objective: To evaluate retrospectively results of treatment of villous adenoma arising from ampulla of Vater with dysplasia or associated carcinoma limited to the ampulla.Patients and Methods: From 1985 to 1996, eight patients have been diagnosed with ampullary villous adenoma suitable for resection. We have reviewed treatment, morbidity, mortality, follow-up and final outcome.Results: Pancreatoduodenectomy (PD) was performed in 4 patients. Transduodenal ampullectomy and endoscopic resection was performed in 2 patients each. There was no perioperative mortality. None of the patients had biliary, pancreatic or intestinal leakage but two patients who underwent PD had minor postoperative complications. The mean follow-up was 44 (range: 6–132) months. Villous adenoma was associated with adenocarcinoma in 50% of the cases (4/8 patients). During the followup both patients who underwent transduodenal ampullectomy developed recurrent disease. All patients initially treated by PD are alive without evidence of recurrent disease.Conclusions: Treatment of villous adenoma of the ampulla must be individualized within certain limits. In our series, PD achieve good results and it appears to be the procedure of choice in order to treat villous adenomas with proved presence of carcinoma, carcinoma in situ or severe dysplasia. Endoscopic or local resection may be appropriate for small benign tumours in high risk patients.     

Lymph Node Involvement and Not the Histophatologic Subtype Is Correlated with Outcome After Resection of Adenocarcinoma of the Ampulla of Vater

Background

Intestinal and pancreaticobiliary types of Vater’s ampulla adenocarcinoma have been considered as having different biologic behavior and prognosis. The aim of the present study was to determine the best immunohistochemical panel for tumor classification and to analyze the survival of patients having these histological types of adenocarcinoma.

Method

Ninety-seven resected ampullary adenocarcinomas were histologically classified, and the prognosis factors were analyzed. The expression of MUC1, MUC2, MUC5AC, MUC6, CK7, CK17, CK20, CD10, and CDX2 was evaluated by using immunohistochemistry.

Results

Forty-three Vater’s ampulla carcinomas were histologically classified as intestinal type, 47 as pancreaticobiliary, and seven as other types. The intestinal type had a significantly higher expression of MUC2 (74.4% vs. 23.4%), CK20 (76.7% vs. 29.8%), CDX2 (86% vs. 21.3%), and CD10 (81.4% vs. 51.1%), while MUC1 (53.5% vs. 82.9%) and CK7 (79.1% vs. 95.7%) were higher in pancreatobiliary adenocarcinomas. The most accurate markers for immunohistochemical classification were CDX2, MUC1, and MUC2. Survival was significantly affected by pancreaticobiliary type (p?=?0.021), but only lymph node metastasis, lymphatic invasion, and stage were independent risk factors for survival in a multivariate analysis.

Conclusion

The immunohistochemical expression of CDX2, MUC1, and MUC2 allows a reproducible classification of ampullary carcinomas. Although carcinomas of the intestinal type showed better survival in the univariate analysis, neither histological classification nor immunohistochemistry were independent predictors of poor prognosis.     

Ampulla of Vater. Anatomic, embryologic, and surgical aspects

The region of the ampulla of Vater constitutes a complex anatomic and functional entity, the biliopancreaticoduodenal confluence, of which the essentials of this rapid review are the: Variation in site of implantation of the greater duodenal papilla, whereas the relations between the common bile duct and the main pancreatic duct are relatively constant Presence at this site of a weak point in the duodenal wall, commonly the site of mucosal diverticula Interdependence of the parietal duodenal mucosa and the sphincteric system of Oddi Existence of an extramural zone of this sphincter, which should be the only one involved in sphincterotomy Danger of wide excisions of the papilla, which, apart from the risk for hemorrhage, cause a breach of the digestive barrier The ampulla of Vater corresponds to the dilated junction of the common bile duct and main pancreatic duct, if present. The ampulla is an extensive anatomic and functional region that includes not only the choledochopancreatic junction but also the sphincter of Oddi, the whole traversing the duodenal wall to open at the greater duodenal papilla. The chief anatomic features of this biliopancreaticoduodenal junction have been reviewed, forming the basis of techniques of surgical or endoscopic sphincterotomies and localized excisions of vaterian tumors.     

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.     

Adenocarcinoma of the Ampulla of Vater: T-Stage,Chromosome 17p Allelic Loss,and Extended Pancreaticoduodenectomy are Relevant Prognostic Factors

Objective To evaluate the prognostic significance of different clinico-pathological and molecular factors, and to compare survival after standard and extended pancreaticoduodenectomy (PD) in ampulla of Vater adenocarcinoma (AVAC). Summary Background Data There are discordant data on factors affecting prognosis, and hence therapeutic choices, in AVAC. Patients and Methods Clinical-pathological factors were evaluated in 59 patients, subjected to PD for AVAC; in 42 subjects information on chromosome 17p and 18q allelic losses (LOH) and microsatellite instability (MSI) was also available. The association between survival and type of PD was investigated in the 25 patients operated between 1990 and 2001 (16 standard and nine extended). Results The overall 5- and 10-year tumor-related survival rates were 46% and 33%, respectively. Sixteen patients had T-stages 1–2, 14 T-stage 3, and 29 T-stage 4 cancers. Chromosome 17p and 18q LOH were detected in 23 (55%) and 15 cases (36%), respectively, and in 12 cases (29%) coexisted. Five cases were MSI-positive (12%). At univariate analysis, poor survival was associated with cancer ulceration (P = 0.051), poor differentiation (P = 0.008), T-stage 4 (P < 0.001), nodal metastases (P = 0.004), chromosome 17p (P < 0.001) and 18q LOH (P = 0.002), and absence of MSI (P = 0.009). At multivariate analysis, only T-stage (P = 0.002) and 17p LOH (P = 0.001) were independent predictors of survival. All patients with MSI-positive cancers were long-survivors (>12 yrs), whereas only 30% of MSI-negative cancer patients survived at 5 years. Extended pancreaticoduodenectomy was associated with a 3-year disease-related survival higher than standard resection (83% vs 31%; P = 0.018). Conclusion MSI and chromosome 17p status allow to better define prognosis within ampullary cancers at the same stage. Surgery alone resulted curative in MSI-positive cancer patients, whereas it was inadequate in patients showing allelic losses, who might benefit from adjuvant therapy. In this observational study, extended PD was associated with increased survival compared to standard procedures. Presented at the 2006 Annual Meeting of the American Hepato-Pancreato-Biliary Association, Miami Beach, Florida, March 9–12, 2006     

Clinical Outcomes for Neuroendocrine Tumors of the Duodenum and Ampulla of Vater:A Population-Based Study

Background

Previous case series report that neuroendocrine tumors (NETs) of the ampulla of Vater have worse overall survival (OS) than NETs in the duodenum. We aimed to compare the OS of patients with ampullary NETs to patients with duodenal NETs.

Methods

This retrospective comparative cohort study used the Surveillance, Epidemiology, and End Results (SEER) registry from 1988 to 2009. OS was evaluated using Kaplan–Meier estimates and Cox proportional hazard regression.

Results

Ampullary NETs (n?=?120) were larger (median size 18 vs. 10 mm, p?<?0.001), higher grade (poorly and undifferentiated tumor 42 % vs. 12 %, p?<?0.001), higher SEER historic stage (distant metastasis 16 % vs. 7 %, p?<?0.001), and more often resected (78 % vs. 60 %, p?<?0.001) than duodenal NETs (n?=?1,360). Median OS was significantly worse for patients with ampullary NETs than with duodenal NETs (98 vs. 143 months, p?=?0.037). Local resection was performed for 50.5 % of the resected ampullary NETs and resulted in similar OS compared to locally resected duodenal NETs (HR 1.37, 95 % CI 0.76–2.48, p?=?0.291).

Conclusions

While ampullary NETs are more advanced at presentation and have worse OS than duodenal NETs, long-term survival is possible with proximal small bowel NETs. For locally resected NETs, OS is similar between ampullary and duodenal NETs.     

Signet-Ring Cell Carcinoma of the Ampulla of Vater: Report of a Case

Signet-ring cell carcinoma (SRCC) of the ampulla of Vater is uncommon, and only 13 cases have been previously described. We herein report a rare case of a 43-year-old woman with SRCC in the ampulla of Vater. Although this histologic type of cancer generally predicts a poor prognosis elsewhere in the gastrointestinal tract, the present patient has survived with no evidence of recurrence for 7.5 years. The patient was hospitalized for pruritus and jaundice. She underwent a pancreaticoduodenectomy with an extended lymphadenectomy for ampullary carcinoma. This neoplasm was classified as AcdbBi, 2.0 x 1.8 cm, ulcerative type, Panc(0), Du(2), T(2)N(0)M(-), Stage II according to the Japanese Classification on Cancer of the Biliary Tract. Unfortunately, previous reports of ampullary SRCC have been limited to short-term follow-ups with a median period of only 12 months (range, 6-134 months), and long-term survival (more than 5 years) was only documented in two cases without lymph node metastasis (including the present case). In conclusion, the experience gained in the present and previous cases suggests that long-term survival is possible following a curative resection in selected patients with ampullary SRCC without nodal involvement. Due to the lack of sufficient evidence, additional reports are warranted to determine whether SRCC also portends a poor prognosis in patients with ampullary cancer.     

Signet-Ring Cell Carcinoma of the Ampulla of Vater: Report of a Case

An 83-year-old man was admitted to our hospital complaining of general fatigue, fever, and obstructive jaundice. Percutaneous transhepatic bile duct drainage was performed. Gastroduodenal fiberscopy revealed carcinoma of the ampulla of Vater, and early gastric cancer was suspected. A pancreatoduodenectomy with lymph node dissection was performed. Although a biopsy specimen from the gastric lesion was suspected to be well-differentiated adenocarcinoma, no cancerous lesion was found in a specimen resected from the stomach. The histopathologic findings of the ampullary lesion were compatible with a diagnosis of signet-ring cell carcinoma. This is a rare lesion, and a review of the literature revealed only three previous similar cases. Received: May 1, 2001 / Accepted: March 5, 2002 RID="*" ID="*" Reprint requests to: N. Eriguchi (address 1)     

壶腹周围癌早期诊断与外科处理(附195例报道)

目的探讨根治性胰十二指肠切除术在壶腹周围癌(AVC)中的临床治疗价值。方法回顾性分析我院1995年3月至2009年3月期间收治的195例AVC患者的临床资料,根据治疗方式分为非手术组(n=51)、姑息性手术组(n=96)和手术切除组(n=48).结果手术切除组术后1、3及5年生存率较姑息性手术组及非手术组明显提高(P0.01),但手术切除组的并发症发生率较姑息性手术组和非手术组明显升高(P0.05).外科治疗的不同部位肿瘤中,胰头癌患者根治切除率较胆总管末段癌及十二指肠乳头癌患者明显降低(P0.01),并且胰头癌患者1、3及5年生存率也明显低于胆总管末段癌及十二指肠乳头癌患者(P0.05,P0.01).术前减黄治疗组与术前未减黄治疗组的围手术期并发症发生率、死亡率及1、3、5年生存率差异均无统计学意义(P0.05).各组中年龄70岁与≤70岁者的并发症发生率差异无统计学意义(P0.05);但手术切除组年龄70岁者的围手术期死亡率明显高于≤70岁者(P0.05).结论外科手术切除仍然是治疗AVC的重要手段,尤其根治性胰十二指肠切除术是治疗壶腹周围癌的惟一有效方法,可以明显延长患者术后存活期,提高其生存质量。     

Sister Mary Joseph Nodule as a Presenting Sign of Pancreatobiliary Adenocarcinoma

Sister Mary Joseph nodules represent metastatic cancer of the umbilicus. These malignancies are usually associated with the ovary and gastrointestinal tract. The authors report the case of a Sister Mary Joseph nodule originating from the bifurcation of the common hepatic duct. Umbilical nodules should prompt clinical evaluations, as these tumors are usually associated with poor prognosis.A 79-year-old man presented to the emergency room with a two-month history of mild periumbilical pain and an enlarging umbilical mass. For the past two weeks, he had experienced worsening right upper quadrant pain and abnormally dark urine, light-colored stool, and yellowing of his skin. Over this same period of time, he experienced anorexia and had lost nearly 15 to 20 pounds. He denied having similar complaints in the past and reported a normal colonoscopy nearly two years earlier.On physical exam, the patient had a 3x3cm hard, firm, ulcerated umbilical nodule with a diffuse infiltration of the entire periumbilical area (Figure 1). He also had right upper quadrant tenderness with a firm liver edge palpated 2cm below the costal margin. Open in a separate windowFigure 1Enlarged ulcerating umbilical tumor with marked deep tissue component     

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.     

Carcinoma of the Ampulla of Vater: Patterns of Failure Following Resection and Benefit of Chemoradiotherapy

Background

Ampullary carcinoma is a rare malignancy. Despite radical resection, survival rates remain low with high rates of local failure. We performed a single-institution outcomes analysis to define the role of concurrent chemoradiotherapy (CRT) in addition to surgery.

Methods

A retrospective analysis was performed of all patients undergoing potentially curative pancreaticoduodenectomy for adenocarcinoma of the ampulla of Vater at Duke University Hospitals between 1976 and 2009. Time-to-event analysis was performed comparing all patients who underwent surgery alone to the cohort of patients receiving CRT in addition to surgery. Local control (LC), disease-free survival (DFS), overall survival (OS), and metastases-free survival (MFS) were estimated using the Kaplan?CMeier method.

Results

A total of 137 patients with ampullary carcinoma underwent Whipple procedure. Of these, 61 patients undergoing resection received adjuvant (n?=?43) or neoadjuvant (n?=?18) CRT. Patients receiving chemoradiotherapy were more likely to have poorly differentiated tumors (P?=?.03). Of 18 patients receiving neoadjuvant therapy, 67% were downstaged on final pathology with 28% achieving pathologic complete response (pCR). With a median follow-up of 8.8?years, 3-year local control was improved in patients receiving CRT (88% vs 55%, P?=?.001) with trend toward 3-year DFS (66% vs 48%, P?=?.09) and OS (62% vs 46%, P?=?.074) benefit in patients receiving CRT.

Conclusions

Long-term survival rates are low and local failure rates high following radical resection alone. Given patterns of relapse with surgery alone and local control benefit in patients receiving CRT, the use of chemoradiotherapy in selected patients should be considered.