Pancreatic invasion is a prognostic indicator after radical resection for carcinoma of the ampulla of Vater

Sixty-three patients who had undergone pancreatoduodenectomy for carcinoma of the ampulla of Vater were analyzed with respect to tumor extent and prognosis. The postoperative mortality rate was 3% and overall survival rates 3 and 5 years after surgery were 55% and 46%, respectively. pTNM stage did not reflect prognosis after resection in patients at stages 2 and 3, while pancreatic invasion and regional lymph node metastasis clearly reflected prognosis after resection. Of the 26 patients who had no pancreatic invasion, regional lymph node metastasis was seen in only 19%, whereas of the 37 patients with pancreatic invasion, 62% exhibited lymph node metastasis. These factors were significantly correlated (P<0.001). Pancreatic invasion appeared to be an indirect indicator of regional lymph node metastasis. We conclude that, to improve prognosis for patients with pancreatic invasion, extended resection including extended lymphadenectomy, is a preferable additional procedure.     

Primary acinar cell carcinoma of the ampulla of Vater

Acinar cell carcinoma of the pancreatobiliary system is a relatively rare malignant neoplasm arising usually in the pancreatic parenchyma. We experienced a 68-year-old woman who presented with obstructive jaundice due to an ampullary mass 1.0 cm in diameter, detected by abdominal computed tomography and endoscopic examination. The patient underwent a curative surgical operation, and histopathological examination revealed that the tumor was confined to the ampulla of Vater with no continuity to the pancreatic parenchyma. The tumor cells showed acinar or tubular arrangement with eosinophilic to basophilic granular cytoplasm, findings identical to those of acinar cell carcinoma of the pancreas. Immunohistochemically, the tumor cells were positive for lipase. From these findings, we concluded that the tumor was primary acinar cell carcinoma arising in the ampulla of Vater, probably originating from heterotopic pancreatic tissue. This is the first reported case of primary acinar cell carcinoma in the ampulla of Vater.     

Feasibility of endoscopic papillectomy in early stage ampulla of Vater cancer

Background and Aim:  Although endoscopic papillectomy has been attempted in early stage ampullary cancer (pTis, T1), its curative role and indications remain uncertain. The present study was designed to assess the factors that predict malignancy and lymph node metastasis and to suggest potential indications for endoscopic papillectomy by analyzing clinicopathological data.
Methods:  We performed a retrospective analysis of clinical and histopathological data of 216 patients with ampullary cancer between 1991 and 2006.
Results:  No tumor in pTis stage had metastasized to lymph nodes and only 9% of tumors in pT1 had metastasized. Tumor size ( P  = 0.018), depth of invasion ( P  = 0.021) and venous invasion ( P  = 0.014) were found to be significantly related to lymph node metastasis. Cases with early stage ampullary cancer of less than 2 cm with a well-differentiated histology and no angiolymphatic invasion ( n  = 13) showed no lymph node metastasis and no recurrence during a median follow up of 35.9 months.
Conclusion:  Endoscopic papillectomy can be adopted as a viable alternative to surgery in patients with early stage ampullary cancer of less than 2 cm in size and with a well-differentiated histology. When a resected specimen has a well-differentiated histology, and there is no resection margin involvement and no angiolymphatic invasion, our findings indicate that subsequent radical surgery is unnecessary.     

Usefulness of follow-up after pancreatoduodenectomy for carcinoma of the ampulla of Vater

Background: The prognosis for carcinoma of the ampulla of Vater (CAV) is better than for pancreatic cancer. The 5-year survival median rate after resection of CAV is 45%, but late recurrences remain possible. Several survival factors have been identified (lymph nodes, perineural invasion), but few data are available on the type of recurrences, their impact and their management. Patients and methods: A total of 41 patients treated by pancreatoduodenectomy (PD) for CAV from 1980 to 2003 were studied retrospectively. Patient selection, long-term survival recurrence rate and recurrence treatment were reviewed. Univariate and multivariate proportional hazards analysis were conducted on this series. Results: The mean follow-up was 48 months. Five-year survival was 62.8%. Eleven patients had recurrences (6–67 months). Recurrence was associated with time to all-causes death (hazard ratio [HR] 4.3, p=0.003). Factors predictive of recurrence were perineural invasion (HR 5.3, p=0.02), lymph node invasion (HR 5.3, p=0.02) and differentiation (HR 0.2, p=0.05). Three patients underwent surgical R0 treatment of their recurrences. Two who presented with solitary liver metastasis are alive and disease-free. Conclusions: Recurrence represents a serious threat in the prognosis of CAV after surgery. Some of these recurrences, in particular liver metastases, are accessible for a curative treatment. This finding supports the usefulness of a close and long-term follow-up after surgery to improve survival of patients with CAV, especially in the group of patients with a good prognosis.     

Large cell neuroendocrine carcinoma of the ampulla of Vater

BACKGROUND: Neuroendocrine tumors of the ampulla of Vater are extremely rare, and few cases of large cell neuroendocrine carcinoma (LCNEC) of the ampulla have been reported. METHODS: A 48-year-old male with obstructive jaundice was admitted to our hospital. On examination the patient was found to have a periampullary growth and subsequently underwent the Whipple's procedure. RESULTS: Histopathological examination and immunohistochemistry revealed features of LCNEC of the ampulla of Vater. The patient developed multiple liver metastases 6 months after Whipple's procedure. CONCLUSION: LCNEC of the ampulla of Vater is rare and highly aggressive, with a dismal prognosis.     

内镜下十二指肠乳头切除术在乳头部肿瘤诊断中的价值

目的:评价内镜下十二指肠乳头切除术(endos-copic papillectomy,EP)在乳头部肿瘤诊断中的临床价值.方法:分析2008-01/2012-03来我院的16例经病理检查确诊的十二指肠乳头部肿瘤患者的相关资料,评价EP在十二指肠乳头部肿瘤诊断中的临床价值.结果:16例患者中,内镜活检诊断为腺瘤者12例,慢性炎症改变者4例.EP将乳头部肿瘤组织切除后整体送检行病理学检查确诊为低分化腺癌者2例,高分化腺癌1例,腺瘤13例(其中1例腺瘤恶变,4例常规内镜活检诊断为慢性炎症改变者术后均确诊为腺瘤),EP术后准确率明显高于内镜活检法(P<0.05).超声内镜(endoscopic ultrasonography,EUS)检查提示病变均起源于黏膜层,胰胆管扩张者9例,未见黏膜下层连续性中断及胰胆管受累者,无腹膜后淋巴结肿大者;12例高回声病变者术后病理检查结果均为腺瘤,1例高回声病变局部见低回声区者术后病理检查结果为腺瘤恶变,3例低回声病变者术后病理检查结果均为腺癌.EP术后2例低分化腺癌者、1例腺瘤恶变者及1例腺瘤者胆管开口处见病变残留,提示胆管受累,但术前EUS检查均未提示胆管受累;1例高分化腺癌及11例腺瘤者病灶均完整切除,术后切缘阴性.EP术后除2例出现黑便外,无急性胰腺炎、穿孔及其他并发症发生,无患者死亡.结论:EP术将乳头部肿瘤组织切除后整体送检行病理学检查可提高诊断准确率,为治疗方法的抉择提供科学依据.     

Trends in incidence and management of cancer of the ampulla of Vater

AIM:To provide trends in incidence,management and survival of cancer of the ampulla of Vater in a welldefined French population.METHODS:Data were obtained from the populationbased digestive cancer registry of Burgundy over a34-year period.Age-standardized incidence rates were computed using the world standard population.Average annual variations in incidence rates were estimated using a poisson regression.A univariate and multivariate relative survival analysis was performed.RESULTS:Age-standardized incidence rates were0.46 and 0.30 per 100000 inhabitants for men and women,respectively.Incidence rate increased from0.26(1976-1984)to 0.58(2003-2009)for men and remained stable for women.Resection for cure was performed in 48.3%of cases.This proportion was stable over the study period.Among cases with curative resection,pancreatico-duodenectomy was performed in94.0%of cases and ampullectomy in 6.0%of cases.A total of 50.8%of cancers of the ampulla of Vater were diagnosed at an advanced stage.Their proportion remained stable throughout the study period.The overall1-and 5-year relative survival rates were 60.2%and27.7%,respectively.Relative survival did not vary over time.Treatment and stage at diagnosis were the most important determinants of survival.The 5-year relative survival rate was 41.5%after resection for cure,9.5%after palliative surgery and 6.7%after symptomatic treatment.In multivariate analysis,only stage at diagnosis significantly influenced the risk of death.CONCLUSION:Cancer of the ampulla of Vater is still uncommon,but its incidence increased for men in Burgundy.Diagnosis is often made at an advanced stage,dramatically worsening the prognosis.     

Carcinoid of the ampulla of Vater： Morphologic features and clinical implications

INTRODUCTION Carcinoid tumors belong to the family of neuroendocrine tumors, which usually grow slowly with distinct biologi- cal and clinical characteristics. The incidence of these tumors is approximately 2.5 in 100 000 people per year. The appendix is …     

Staging of carcinoma of the pancreas and ampulla of Vater

Summary Between 1984 and 1987, 472 Norwegian patients with histologically or cytologically verified carcinoma of the pancreas (N=442) and ampulla of Vater (N=30) were accrued and TNM staged according to UICC. The influence of the T, N, and M categories on long-term survival was evaluated. The T1a and T1b tumors of stage I pancreatic carcinoma had a comparable survival (p=0.68–0.95). A higher T category (T1–T3) predicted a more dismal prognosis (p=0.000). The T1 and T2 carcinomas of the ampulla of Vater had a comparable favorable prognosis, and the T3 and T4 tumors had a comparable unfavorable prognosis. The N1 vs N0 (p=0.000–0.01) and M1 vs M0 categories (p=0.00–0.003) predicted a more dismal prognosis for both pancreatic and ampullary carcinoma. By logistic regression analyses, pancreatic tumor extension into peripancreatic fat or nerves and invasion of ampullary carcinomas into duodenal wall, unfavorably influenced the N1 category (p=0.000–0.04) and tumor diameter influenced the M1 category (p=0.002–0.04) both for pancreatic and ampullary carcinoma. The T, N, and M categories all independently influenced survival of pancreatic carcinoma (p=0.000–0.003). Only the N category (p=0.01) influenced the prognosis of ampullary carcinomas.     

KL-6 mucin expression in carcinoma of the ampulla of Vater： Association with cancer progression

AIM: To assess histochemical expression of KL-6 and its clinicopathological significance in carcinoma of the ampulla of Vater. METHODS: Ampullary carcinoma tissues were collected from 38 patients who underwent pancreatoduodenectomy or local resection. Tissues were subjected to immunohistochemical analysis using KL-6 antibody. RESULTS: Positive staining of ampullary carcinoma cells was observed in 26 (68.4%) cases. Staining was not found in the surrounding non-cancer regions of the ampullary tissues. Remarkable KL-6 expression was observed in invasive carcinoma cells in pancreatic and duodenal tissues and in metastatic carcinoma cells in lymph nodes. Positive KL-6 expression was related to lymph node metastasis (P=0.020), pancreatic invasion (P=0.016), duodenal invasion (P= 0.034), and advanced stage of TNM clinical classification (P=0.010). Survival analysis showed that positive expression of KL-6 was related to a poorer prognosis (P=0.029). CONCLUSION: The aberrant expression of KL-6 mucin is significantly related to unfavorable behaviors of carcinoma of the ampulla of Vater.     

Cancer of the ampulla of Vater: chromosome 17p allelic loss is associated with poor prognosis

BACKGROUND: Cancer of the ampulla of Vater kills 60% of affected patients. Local spread of the tumour (T stage) is the only reliable prognostic factor. Nevertheless, any cancer stage includes long term survivors and patients dying from the disease. The molecular anomalies involved in this process have the potential to serve as additional prognostic markers. AIM: To evaluate if allelic losses (LOH) of chromosomes 17p and 18q may be of prognostic value in multivariate survival analysis. METHODS: We examined 53 ampullary cancers for chromosome 17p and 18q LOH using microsatellite markers and DNA from paraffin embedded tumours. All patients were treated by surgery alone (pancreaticoduodenectomy). Multivariate survival analysis included age, sex, tumour size, macroscopic appearance, grade of differentiation, T stage, lymph node metastasis, and chromosome 17p and 18q status. RESULTS: Chromosome 17p and 18q LOH were detected in 28 (53%) and 18 (34%) cancers, respectively. Multivariate survival analysis indicated chromosome 17p status as an independent prognostic factor together with T stage. The five year survival for chromosome 17p retention and 17p loss was 80% and 7%, respectively. The risk of death from cancer within the five year follow up period for patients with cancers harbouring chromosome 17p LOH was 11 times higher than that of patients with cancers retaining chromosome 17p (p<0.0001), regardless of the tumour stage at diagnosis. CONCLUSIONS: Chromosome 17p status is an independent prognostic factor among ampullary cancers at the same stage. The combined use of T stage and chromosome 17p status may help in deciding whether ampullary cancer patients require additional therapy other than surgery alone.     

Small-cell neuroendocrine carcinoma of the ampulla of Vater

We report a patient (an 80-year-old woman) with anemia and fecal occult blood, who had an emergency operation for carcinoma of the cecum (well-differentiated adenocarcinoma without local lymph node metastasis). Postoperative magnetic resonance cholangiopancreatography, cholangiography, and upper gastroduodenal endoscopy showed a tumor of the ampulla of Vater, and pylorus-preserving pancreatoduodenectomy was performed. Histology of the resected tumor was that of small-cell carcinoma, and immunohistochemistry showed positive staining for neuron-specific enolase, chromogranin A, and synaptophysin, confirming the neuroendocrine nature of the tumor. As the histology of the tumor was distinct from cecal carcinoma, and no tumors were found in other organs, the tumor was diagnosed as primary small-cell neuroendocrine carcinoma of the ampulla of Vater. The patient died due to multiple liver metastases of the carcinoma of the ampulla of Vater 7 months after the pancreatoduodenectomy. The clinical and morphological features of this disease have been reported in nine individuals previously.     

A case of gastrointestinal stromal tumour of the ampulla of Vater

Gastrointestinal stromal tumour rarely develops in the duodenal ampulla region. We report here a case of gastrointestinal stromal tumour of the ampulla of Vater found in a 44-year-old Japanese man presenting with biliary obstruction. He died of hepatic failure with diffuse liver metastasis. The postmortem examination showed a large Borrman type III-like tumour in the duodenal ampullary region with direct invasion of the pancreas and extrahepatic bile duct as well as metastases to the liver and regional lymph nodes. The duct orifice was located at the centre of the tumour. Microscopically, the tumour consisted of anaplastic spindle cells with high mitotic activity (90 mitoses per 50 high-power fields). Immunohistochemically, the spindle cells were positive for KIT and CD34. The final diagnosis was high-grade malignant gastrointestinal stromal tumour of the ampulla of Vater. Considering the recent advances in the diagnosis and treatment of gastrointestinal stromal tumour, this neoplasm should be included in the differential diagnosis of the tumours appearing in the duodenal ampulla region.     

经内镜十二指肠乳头切除术治疗乳头部肿瘤的临床价值

目的 探讨经内镜十二指肠乳头切除术(EP)治疗十二指肠乳头部肿瘤的临床价值.方法 前瞻性观察研究15例经EP治疗的十二指肠乳头部肿瘤患者的临床疗效、并发症及随访观察结果,评价其临床价值.结果 所有15例患者均顺利完成EP治疗,其中4例术前病检为慢性炎症者术后病理诊断为腺瘤;11例术前病检为腺瘤者中术后病理诊断为高分化腺癌1例、低分化腺癌2例、腺瘤恶变1例.EP术后8例合并胰胆管扩张者同时行胆管及胰管塑料支架引流术,1例确诊为低分化腺癌者、1例确诊为腺瘤恶变者及1例腺瘤部分残留者术后追加外科手术治疗,另1例低分化腺癌者因年龄较大拒绝追加手术治疗.EP术后除2例患者出现黑便,2例患者出现一过性血淀粉酶升高外,无急性胰腺炎、胆管炎及穿孔等并发症发生,无患者死亡.EP术后平均随访观察23.4个月,11例(11/15,73.3％)病变完整切除者中,10例腺瘤者均未见复发,另1例乳头部高分化腺癌者术后已随访5个月,多次复查病检均未见复发.结论 EP术不仅可提高十二指肠乳头部肿瘤诊断的准确率;对于十二指肠乳头部腺瘤,EP术可将腺瘤组织完整切除达到根治的目的,是一项安全、有效的微创治疗措施.     

Signet-ring cell carcinoma of ampulla of Vater： Contrast-enhanced ultrasound findings

Signet-ring cell carcinoma （SRCC） of ampulla of Vater is extremely uncommon, and less than 15 cases have been reported so far in literature. It mainly occurs in elderly people （median age 57 years）. We report a rare case of SRCC of the ampulla of Vater in a 38-year-old woman who presented with a small tumor at the Vater, discovered by the contrast-enhanced ultrasound （CEUS）. Histopathological examination showed prominent signet-ring features. We also describe the imaging features of SRCC of ampulla of Vater in CEUS.     

Efficacy of cap-assisted endoscopy for routine examining the ampulla of Vater

AIM: To determine the efficacy of a cap-assisted endoscopy (CAE) to completely visualize the ampulla of Vater (AV) in patients failed by conventional endoscopy. METHODS: A prospective study was conducted on 120 patients > 20 years of ages who visited the Health Promotion Center of Chungbuk National University Hospital for conscious sedation esophagogastroduodenoscopy (EGD) as a screening test from July to October, 2011. First, forward-viewing endoscopy was performed with reasonable effort using a push and pull method. We considered complete visualization of the AV when we could observe the entire AV including the orifice clearly, and reported the observation as complete or incomplete (partial or not found at all). Second, in cases of complete failure of the observation, an additional AV examination was conducted by attaching a short cap (D-201-10704, Olympus Medical Systems, Tokyo, Japan) to the tip of a forward-viewing endoscope. Third, if the second method failed, we replaced the short cap with a long cap (MH-593, Olympus Medical Systems) and performed a re-examination of the AV. RESULTS: Conventional endoscopy achieved complete visualization of the AV in 97 of the 120 patients (80.8%) but was not achieved in 23 patients (19.2%). Age (mean ± SD) and gender [male (%)] were not significantly different between the complete observation and the incomplete observation groups. Additional short CAE was performed in patients in whom we could not completely visualize the AV. This group included 13 patients (10.9%) with partial observation of the AV and 10 (8.3%) in which the AV was not found. Short CAE permitted a complete observation of the AV in 21 of the 23 patients (91.3%). Patients in whom visualization of the AV failed with short CAE had satisfactory outcomes by replacing the short cap with a long cap. The additional time for CAE took an average of 141 ± 88 s. There were no complications and no significant mucosal trauma. CONCLUSION: CAE is safe to use as a salvage method to achieve complete visualization of     

Malignant carcinoid tumor in the papilla of Vater: case report and review of 25 cases reported in Japan

Carcinoid tumors in the papilla of Vater are rare. We describe a 48-year-old male who was diagnosed with a tumor consisting of atypical cells in the papilla of Vater. He underwent curative resection of the tumor by pancreatico-duodenectomy (PD), with dissection of the regional lymph nodes. Microscopic examination revealed a metastatic lymph node in the pancreatic region. Characteristic findings on histopathological and electron microscopic studies led to the final diagnosis of malignant carcinoid tumor. It is clear from the literature that carcinoid tumors in the papilla of Vater metastasize to the regional lymph nodes and the liver. However, it has been difficult to preoperatively diagnose carcinoid tumor in the papilla of Vater. Therefore, if we observe atypical cells in the papilla of Vater, malignant carcinoid tumor should be considered. If this tumor is suspected, PD or pylorus-preserving PD, together with dissection of the regional lymph nodes, at least along the hepatoduodenal ligament, should be considered as the treatment of first choice.     

Allelotype of ampulla of Vater cancer: highly frequent involvement of chromosome 11

Purpose To determine the genetic differences/similarities in ampulla of Vater cancers (AVC) with respect to other pancreatic tumor types.Methods We analyzed eight cases of primary AVC by genome-wide allelotyping on DNA obtained from frozen tissue. A total of 372 microsatellite loci were used for each case, for a total of 2,976 microsatellites analyzed.Results Of the 2,159 informative markers, 400 were allelic losses and 1,759 markers were retained for an average fractional allelic loss of 0.19. Seven cases showed LOH on at least two markers on chromosomal arm 11p, while six cases showed allelic losses on 11q. The high frequency of LOH on chromosome 11 was also confirmed by analysis of an additional 17 paraffin-embedded AVC. Frequent LOH (50% or greater) was also found on chromosome arms 5q, 6q, 9p, 13, 16p, 17p, and 18p.Conclusions It can be inferred that the targets of inactivation on chromosomes 5q, 9p, and 17p appear to be APC, p16, and p53, respectively, while the critical target(s) of inactivation at the other frequently lost loci remain to be characterized. The resulting allelotype reveals that distinctive chromosomal alterations are present in these neoplasms, indicating that it is a tumor entity distinct from pancreatic adenocarcinoma.     

Signet ring cell carcinoma of the ampulla of Vater

Introduction

Signet ring cell carcinoma of the ampulla of Vater is a rare entity and less than 20 cases have been described in the literature. We report the cases of two patients with this disease and provide a literature review of previous studies.

Case report

We describe two patients with obstructive jaundice. Abdominal ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic and common bile duct. Duodenoscopy indicated a protruding mass on the ampulla of Vater. Histopathological examination showed round cells and their nuclei were located on one side with prominent signet-ring features. One patient underwent a cephalic pancreatoduodenectomy with lymphadenectomy and the other a total pancreatectomy.

Discussion

Signet ring cell carcinoma of the ampulla of Vater has only been described in isolated cases in the literature. Therefore, the clinicopathological features and prognosis of this disease have not yet been well defined.