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 cardnoma of the ampulla of Vater.

Prognostic relevance of number and ratio of metastatic lymph nodes in resected carcinoma of the ampulla of Vater

Objectives： To explore the prognostic relevance of the number and ratio of metastatic lymph nodes in resected Carcinoma of the ampulla of Vater （CAV）. Methods： The clinical data of 155 patients who underwent pancreaticoduodenectomy （PD） for cancer of the ampulla of Vater between January 1990 and December 2010 were retrospectively analyzed. Kaplan- Meier method was used in survival analysis and Log rank method in comparison. Multivariate analysis was performed using Cox proportional hazards model. Results： Among these 155 patients, the in-hospital mortality rate was 4.5%, lymph node positive disease was 21.3%, and the 5-year survival rate was 51.6%. Patients with a lymph node ratio （LNR） 〉20% were more likely to have tumor differentiation, depth of duodenal involvement, depth of pancreatic invasion, T-stage and TNM-Stage. The number of the metastatic lymph nodes is important prognostic factors of the CAV. Univariate analysis showed that the factors associated with the prognosis included tumor size （P=0.036）, tumor differentiation （P=0.019）, LNR （P=0.032）, mtmber of metastatic lymph nodes （P=0.024）, lymph node metastasis （P=0.03）, depth of pancreatic invasion （P=0.001）, T-stage （P=0.002）, TNM stage （P=0.001）, elevated CA 19-9 （P=0.000）, and jaundice （P=0.021）. Multivariate analysis showed that the factors associated with the prognosis were the number of metastatic lymph nodes （P=0.032; RR： 1.283; 95% CI： 1.022-1.611）, tumor size （P=0.043; RR： 1.736; 95% CI： 1.017-2.963）, and elevated CA 19-9 （P=0.003; RR： 3.247; 95% CI： 1.504-7.010）. Conclusions： LNR is a useful factor for predicting the prognosis of the radical treatment for CAV,, whereas the number of metastatic lymph nodes is the most important factor. Further research on the locations, number, and LNR will be clinically meaningful to improve survival in patients with CAV.

Coexistence of small cell neuroendocrine carcinoma and villous adenoma in the ampulla of Vater

Small cell neuroendocrine carcinoma of the ampulla of Vater is extremely rare and different from the common ampullary adenocarcinoma. The ampullary adenoma is also a rare neoplasm and has the potential to develop an adenocarcinoma. Their coexistence has been rarely reported in the literature. We herein describe an unusual case of a small cell neuroendocrine carcinoma associated with a villous adenoma in the ampulla of Vater with emphasis on computed tomography (CT) and histopathological findings. We also discuss their clinical, histopathological and radiological features as well as possible histogenesis.

Rare histological subtype of invasive micropapillary carcinoma in the ampulla of Vater: A case report

BACKGROUND Carcinoma of the ampulla of Vater is an uncommon ampullo-pancreatobiliary neoplasm,and the most common histological type is adenocarcinoma with a tubular growth pattern.Invasive micropapillary carcinoma(IMPC)is an aggressive variant of adenocarcinoma in several organs that is associated with lymph node metastasis and poor prognosis.IMPC was first described as a histological subtype of breast cancer;however,IMPC of the ampulla of Vater is extremely rare,with only three articles reported in the English literature.CASE SUMMARY We have reported a case of IMPC of the ampulla of Vater in an 80-year-old man.Microscopically,the surface area of the carcinoma was composed of tubulopapillary structures mimicking intra-ampullary papillary-tubular neoplasm,and the deep invasive front area exhibited a pattern of IMPC.The carcinoma showed lymphatic invasion and extensive lymph node metastasis.The immunohistochemical study revealed mixed intestinal and gastric/pancreatobiliary phenotypes.CONCLUSION This rare subtype tumor in the ampulla of Vater showed a histologically mixed phenotype and exhibited aggressive behavior.

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.

Role of adjuvant chemotherapy on recurrence and survival in patients with resected ampulla of Vater carcinoma

BACKGROUND Owing to rarity of disease and lack of prospective studies, data supporting the role of adjuvant chemotherapy in ampulla of Vater(AoV) carcinoma is limited.AIM To evaluate whether adjuvant chemotherapy cases for AoV carcinoma had better disease-free survival(DFS) rates than cases of observation following curative surgery.METHODS We retrospectively analyzed the association between adjuvant chemotherapy and DFS and overall survival(OS) in patients with stage IB-Ⅲ AoV carcinoma who underwent curative surgical resection. Fluorouracil-based adjuvant chemotherapy was administered after surgery at the discretion of the physician. Adjusted multivariate regression models were used to evaluate the association between adjuvant chemotherapy and survival outcomes.RESULTS Of the total 104 patients who underwent curative surgery, 52 received adjuvant chemotherapy. Multivariate analysis revealed that higher histologic grade [hazard ratio(HR) = 2.24, P = 0.046], advanced tumor stage(HR = 1.85, P = 0.030), and vascular invasion(HR = 2.14, P = 0.010) were associated with shorter DFS. Adjuvant chemotherapy improved DFS compared to the observation group(HR =0.50, P = 0.015) and tended to be associated with a longer OS, although the difference was not statistically significant(HR = 0.58, P = 0.098).CONCLUSION Among patients with resected AoV carcinoma, the adjuvant chemotherapy group was not associated with a significant survival benefit compared to the observation group. However, on multivariate analysis adjusting for prognostic factors, adjuvant chemotherapy following surgery was an independent prognostic factor for DFS in patients with resected AoV carcinoma. Further studies are needed to investigate the effectiveness of adjuvant chemotherapy according to histologic phenotype.

A rare case of periampullary carcinoma with ectopic ending of Vater's ampulla

A 71-year-old woman was referred to our department complaining of painless progressive jaundice for the last 3 too. Magnetic resonance imaging and magnetic resonance cholangiopancreatography （MRCP） showed the ectopic hepatopancreatic ampulla draining into the fourth part of the duodenum adjacent to the duodenojejunal flexure; the irregular morphology of the duodenojejunal flexure likely due to a soft tissue mass. Laparotomy confirmed the presence of the abnormal ampulla of Vater located at the fourth part of the duodenum and a soft tissue tumor about 6 cm × 5 cm×5 cm with a peduncle adjoining the ampulla. Resection of the tumor, including some peripheral tissue, and a Roux-Y loop anastomosis choledochojejunostomy were performed. Pathological examination indicated an intestinal villous adenoma accompanied by severe dysplasia and focal canceration. Periampullary carcinoma with ectopic ending of the Vater＇s ampulla into the fourth part of the duodenum is rather rare. The embryonic genetic background of this anomaly has not yet been fully explained. It is worth mentioning that MRCP is useful for demonstrating anomalies and anatomic variants of the biliary tract system and pancreatic duct.

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.

Prognostic Factors of Ampulla of Vater Carcinoma after Radical Surgery

OBJECTIVE Ampullary carcinoma is a rare disease with betterprognosis than other periampullary neoplasms. This studyinvestigated the association between clinicopathologic factors andprognosis after radical resection of ampulla of Vater carcinoma.METHODS Clinical data from 105 patients who underwentradical pancreaticoduodenectomy from January 1990 to December2005 were retrospectively analyzed by the Kaplan-Meier method,log-rank test, and the Cox proportional hazard model.RESULTS The in-hospital mortality rate was 8.6%, the lymphnode metastasis rate was 37.1%, and the five-year survival ratewas 42.8%. Pancreatic involvement (P = 0.027), tumor diameter (P= 0.008), T stage (P = 0.003), TNM stage (P < 0.001), and number ofmetastatic lymph nodes (P < 0.001) were associated with prognosiswhen the univariate analysis was used. Multivariate analysisshowed that the number of lymph node metastases (P < 0.001;OR: 1.923; CI: 1.367-2.705) and tumor diameter (P = 0.03; OR: 1.432;CI: 1.035-1.981) were the independent prognostic factors.CONCLUSION The number of metastatic lymph nodes andtumor diameter are important pathologic factors predictingprognosis of ampulla of Vater carcinoma after radical resection,and lymph node dissection during the radical surgery effectivelyimproves the survival rate.

Signet Ring Cell Carcinoma of Ampulla of Vater: Report of a Rare Malignancy and Literature Review

Introduction: Signet ring cell carcinoma is a rare tumor that generally originates in the gastrointestinal tract. Signet-ring cell carcinoma of the ampulla of Vater is extremely uncommon and a very rare clinical entity, which is infrequently reported in medical literature and only 31 cases have been mentioned. Most tumors affecting Vater’s Ampulla are adenocarcinomas and other histological variants are less frequent. It mainly occurs in elderly patients. Case Presentation: We report a case of signet ring cell carcinoma of Vater’s Ampulla. The tumour had infiltrated the duodenal, but local lymph nodes were clear (T3N0M0). Duodenopancreatectomy with pylorus preservation is the treatment of choice. Conclusions: Etiology and survival are not well-defined in the literature due to the extreme rarity of this disease.

Intra-ampullary papillary-tubular neoplasm combined with ampullary neuroendocrine carcinoma:A case report

BACKGROUND The ampulla of Vater is an anatomically and histologically complex region giving rise to a heterogenous group of tumors.This is,to the best of our knowledge,the first case of intra-ampullary papillary-tubular neoplasm combined with ampullary neuroendocrine carcinoma reported in the literature.CASE SUMMARY A 61-year-old woman presented to the emergency department for evaluation of painless jaundice.Contrast-enhanced computed tomography(CT)of the abdomen and chest showed a periampullary tumor mass measuring 15 mm×12 mm×14 mm,with no evidence of locoregional and distant metastases,for which she underwent pancreatoduodenectomy.Histopathologic examination of a resected specimen revealed an intra-ampullary papillary tubular neoplasm with highgrade dysplasia in combination with poorly differentiated grade 3 neuroendocrine carcinoma with a mitotic count of more than 20 mitoses per 10 high power fields and Ki-67 index of 100%.No positive lymph nodes were identified.Her postoperative course was uneventful.Postoperatively,she remained under close surveillance.Multiple liver metastases were observed on follow-up CT 8 mo after the surgery,so systemic therapy with cisplatin and etoposide was initiated.CONCLUSION The simultaneous occurrence of neuroendocrine and non-neuroendocrine tumors in the ampulla of Vater is rare and the pathogenesis of such tumors is largely unknown.Due to unpredictable clinical behavior and lack of solid evidence on optimal treatment strategy,close patient surveillance is advised after radical resection of the primary tumor.

Jaundice as a prognostic factor in patients undergoing radical treatment for carcinomas of the ampulla of Vater

Background Carcinomas of the ampulla of Vater （CAV） is a relatively rare malignant gastrointestinal tumor,and its postoperative prognostic factors have been well studied.However,as its first symptom,the impact of jaundice on the prognosis of CAV is not so clear.This study aims to explore the role of jaundice as a prognostic factor in patients undergoing radical treatment for CAV.Methods The clinical data of 195 patients with CAV who were treated in the Cancer Hospital,Chinese Academy of Medical Sciences ＆ Peking Union Medical College,from January 1989 to January 2013 were retrospectively analyzed.Among them,170 patients with pathologically confirmed CAV entered the statistical analysis.Jaundice was defined as a total bilirubin serum concentration of ≥3 mg/dl.Result Of these 170 patients,99 （58.20％） had jaundice at presentation.Jaundice showed significant correlations with tumor differentiation （P=0.002）,lymph node metastasis （P=0.016）,pancreatic invasion （P=0.000）,elevated preoperative CA199 （P=0.000）,depth of invasion （P=0.000）,and tumor stage （P=0.000）.There were more patients with pancreatic invasion in the jaundice group than in the non-jaundice group.Also,lymph node metastasis was more common in the jaundice group （n=26） than in the non-jaundice group （n=8）.The non-jaundice group had significant better overall 5-year disease-free survival （72.6％） than the jaundice group （41.2％,P=0.013）.Jaundice was not significantly correlated with the postoperative bleeding （P=-0.050）.Conclusions Jaundice in patients with CAV often predicts more advanced stages and poorer prognoses.Pancreatic invasion and lymph node metastasis are more common in CAV patients with jaundice.Jaundice is not a risk factor for postoperative bleeding and preoperative biliary drainage cannot reduce the incidence of postoperative complications.

壶腹癌胰十二指肠切除术后预后影响因素的Meta分析

目的通过综合评价总结出影响壶腹癌预后的因素,为壶腹癌患者的预后判断及个性化治疗提供依据。方法对符合入选标准的1995年至今公开发表的关于壶腹癌术后生存分析的研究进行Meta分析,并计算每个危险因素的优势比值(OR值)以及其95%可信区间(95%CI)。结果共纳入文献13篇,累积病例986例。局部浸润(浸润至胰腺、浸润至十二指肠壁、浸润至Oddi括约肌外)、淋巴结转移、输血、肿瘤分化程度对壶腹癌术后5年死亡率影响的合并后P值及OR值分别为P<0.001,OR=4.74、P=0.01,OR=2.75、P<0.001,OR=4.25、P<0.001,OR=4.16、P<0.001,OR=2.56、P=0.002,OR=2.38。结论肿瘤局部浸润(浸润至胰腺、浸润至十二指肠壁、浸润至Oddi括约肌外)、淋巴结转移、输血、肿瘤分化程度均为壶腹癌预后影响因素。

胆汁CEA、CA19-9联合检测对肝外胆管癌及壶腹癌的诊断价值

目的探讨胆汁CEA、CA19-9联合检测对肝外胆管癌及壶腹癌诊断价值。方法观察组38例肝外胆管癌及壶腹癌患者在行ERCP检查前检测血清CEA及CA19-9,行ERCP检查时经造影导管抽取胆汁5ml用放免方法检测CEA及CA19-9,同时在导丝引导下自胆管咬取活检行病理检查,并记录影像学特征;同样,对照组30例非恶性病变患者在行ERCP检查时抽取5ml胆汁检测CEA及CA19-9。结果观察两组患者胆汁CA19-9、CEA水平明显高于对照组,有统计学意义(P<0.05),与病理结果一致;观察组患者胆汁及血清CA19-9水平比较,有统计学差异(P<0.05),而CEA水平无明显差异(P>0.05)。结论胆汁CEA、CA19-9联合检测对肝外胆管癌及壶腹癌的定性诊断有重要临床价值。

139例壶腹周围癌诊治分析

目的:探讨壶腹周围癌的诊断方法和手术治疗以及影响手术切除率的因素。方法:对139例壶腹周围癌患者的临床资料进行回顾性分析,其中胰头癌109例,壶腹部癌30例。B超、CT、CT血管造影(CTA)、核磁共振成像(MRI)和磁共振胰胆管造影术(MRCP)、经皮肝胆管造影术(PTC)和内窥镜逆行胆总管胰腺造影术(ERCP)检查分别为136、76、73、19、17例。行胰十二指肠切除术43例,胆囊空肠吻合术20例,胆囊十二指肠吻合术3例,胃空肠吻合术19例,胆囊造瘘术4例,胆总管十二指肠吻合术4例,胆总管空肠吻合术21例,胆总管探查、T管引流18例,剖腹探查术7例。结果:壶腹周围癌患者行B超、CT/CTA、MRI/MRCP、PTC和ERCP检查,术前临床诊断准确率分别为81.6%(111/136)、89.3%(68/76)、93.2%(68/73)、68.4%(13/19)、83.2%(15/17),术前评价肿瘤切除准确性分别为71.9%(41/57)、84.8%(38/46)、94.3%(32/35)、63.6%(7/11)、66.7%(8/12)。总手术切除率为30.9%(43/139),胰头癌与壶腹部癌手术切除率分别为23.9%(26/109)与56.7%(17/30),根治性手术死亡率为9.3%(4/43),手术并发症率37.2%(17/43)。结论:腹部非特异性症状结合影像学检查可提高壶腹周围癌的早期诊断率;术前准确评估手术可切除性,制订合适的手术方案,以提高壶腹周围癌的手术切除率,降低手术死亡率、并发症发生率。

壶腹周围癌合并糖尿病患者的围手术期处理

目的 探讨壶腹周围癌合并糖尿病患者的围手术期处理方法。方法 回顾性总结１９９６～１９９８ 年收治的２９ 例壶腹周围癌合并糖尿病患者的临床资料。结果 全部病例均手术治疗，其中胰十二指肠切除术１９ 例，均严格按糖尿病处理，术前血糖控制在５．３～７．６ｍ ｍ ｏｌ／ Ｌ。围手术期疗效满意率为１００％ ，无手术死亡。结论 加强围手术期的处理可提高壶腹周围癌合并糖尿病患者的手术耐受性，降低术后并发症。

超声结合血清学指标在壶腹癌与胰头癌鉴别诊断中应用

目的探讨超声与血清学检查联合应用在壶腹癌与胰头癌鉴别诊断中的价值。方法选择2013年2月至2018年10月在福建医科大学附属协和医院住院手术经病理检查证实的壶腹癌49例,其中男性29例,女性20例;年龄39~77岁,平均年龄59.1岁。经病理检查证实胰头癌63例,其中男性38例,女性25例,年龄35~79岁,平均年龄60.6岁。外科手术前1周内行腹部超声检查和血液肿瘤标记物Ca19-9、血生物化学检查[总胆红素(TBiL)、直接胆红素(DBiL)、天冬氨酸转氨酶(AST)、碱性磷酸酶(ALP)],分析两组患者的血清学指标和超声声像图特征。结果壶腹癌与胰头癌均多见于中老年男性,胰头癌组Ca19-9>200 U/mL所占比率高于壶腹癌组,而壶腹癌组TBiL、DBiL、AST、ALP测值升高的比率高于胰头癌组。壶腹癌组病灶平均最大径(2.42 cm)小于胰头癌组(3.92 cm),壶腹癌组发生肝内外胆管扩张的比率(95.9%)高于胰头癌组(69.8%)。壶腹癌与胰头癌多呈较均质的低回声病灶,乏血液供应,但胰头癌病灶形态更不规则,边界不清,常侵犯周围血管组织。术前超声对壶腹癌与胰头癌的诊断准确率分别为46.94%、100.00%。结论壶腹癌与胰头癌在超声表现与血液学指标方面存在一定的差异,超声检查时应综合分析判断,对于提高术前诊断率,为临床制定诊疗方案具有重要意义。

AFP阳性胰腺癌和壶腹乳头部癌的临床病理研究

目的:研究AFP阳性胰腺癌(4例)和壶腹乳头部(1例)癌的临床病理特征.方法:收集并分析5例此癌患者的临床资料,对其病理标本应用光镜、免疫组织化学进行研究.结果;此类癌多见于中老年人,血清AFP明显增高;5例均发生肝转移,于1年内死亡,平均生存期5个月;病理组织形态:均为浸润性癌,脉管内常见癌栓;2例为分化差腺癌,2例为肝样腺癌(1例为壶腹乳头部,1例为胰腺),后者癌组织由两种不同而又密切相关的腺癌区和肝样分化区组成,肝样区癌细胞具有与肝细胞癌相类似的形态特征,其癌细胞AFP染色呈阳性表达.结论:此类癌由于AFP具有免疫抑制作用,癌细胞侵袭性强易发生肝转移,病人预后不良,属高度恶性肿瘤.

十二指肠壶腹部神经内分泌癌的诊断与治疗（附七例报告）

目的 探讨十二指肠壶腹部神经内分泌癌临床病理学特征、诊断和治疗方法.方法 回顾分析7例十二指肠壶腹部神经内分泌癌病例并结合文献,综合分析十二指肠壶腹部神经内分泌癌的临床病理学特征、诊断及治疗.结果 7例十二指肠壶腹部神经内分泌癌占同期十二指肠壶腹部肿瘤的4.32%（7/162）.主要临床症状为腹痛、梗阻性黄疸、消化道出血和贫血.术前影像学检查是诊断十二指肠壶腹部肿瘤的主要手段,其中1例患者在术前经内镜及内镜超声确诊.经组织病理及免疫组化诊断为大细胞神经内分泌癌3例、小细胞神经内分泌癌3例,不典型类癌1例.其中行胰十二指肠切除术5例,行十二指肠乳头部肿瘤局部切除术2例.所有患者均得到随访.全组中位生存期为23个月.结论 十二指肠壶腹部神经内分泌癌罕见.针对十二指肠壶腹部高级别神经内分泌癌进行根治性切除可能改善患者的预后.

磁共振胆胰管成像对壶腹部肿瘤诊断的价值

目的 评价磁共振胆胰管成像对壶腹部癌的诊断价值。方法 通过总结 2 3例壶腹部癌的 MRCP及 MRI影像资料 ,对磁共振影像诊断与病理学诊断的符合率进行回顾性分析。结果 磁共振胆胰管成像的定位诊断率为 10 0 % ,与病理诊断的符合率也达 91.3%。结论 磁共振胆胰管成像是一种非介入性胆胰管成像技术 ,具有不需要对比剂、无创伤及图像清晰的优点。