Endoscopic resection for neoplastic diseases of the papilla of Vater

Optimal management of neoplastic diseases of the papilla of Vater is still controversially discussed. Until a few years ago, surgical resection or transduodenal local excision were routinely performed as definitive treatments. In order to decrease mortality and morbidity, investigators systematically started in the late 1980s to evaluate alternative methods, particularly following an endoscopic strategy. In recent years, endoscopic resection procedures (e.g., snare resection, piecemeal resection, thermal ablative techniques) proved to be feasible and safe alternatives, especially for benign neoplastic diseases of the papilla. The following review summarizes criteria for the selection of patients and describes endoscopic resection techniques. In addition, outcome concerning recurrence rates and complications of surgical and endoscopic resection procedures are evaluated.     

Distended glands or overreplacement of ampullary mucosa at the papilla of Vater

Background/Purpose The role of the ampullary mucosa, especially its distended glands at the papilla of Vater, has not been fully explored.Methods Twenty-nine pancreatoduodenectomized specimens from pancreatobiliary diseases and 44 autopsied cases, as controls, were studied histopathologically and immunohistochemically.Results In 12 out of the 29 pancreatoduodenectomized cases the ampullary mucosa was in contact with the duodenal mucosa just at the outlet of the ampulla. In the remaining 17 cases, the ampullary mucosa overgrew beyond the ostium, replacing a portion of the surrounding duodenal mucosa, termed distended glands, which measured an average of 1532µm in length. The muscularis mucosae of the duodenum and the Oddis sphincter muscle merged in an end-to-end, sharp-angled manner at the ostium in the former, whereas this occurred in an end-to-side, less sharp, rather right-angled manner in the latter. Immunohistochemically, the distended glands in some cases showed negative/weakly positive staining for anti-carbohydrate antigen (CA) 19-9 and a high proliferation index evaluated using Ki67. In the autopsied materials, distended glands were found in 24 out of the 44 cases.Conclusions Distended glands of the ampullary mucosa were frequently found and only grew on the Oddis sphincter muscle extension. They may represent not only malignant change but also an adaptive phenomenon for bile and pancreatic juice flow.     

Long-term survival after hepatectomy for hepatic recurrence of carcinoma of the papilla of Vater

We present herein a rare case of a long-term survivor after major hepatectomy performed for a metastatic liver tumor from carcinoma of the papilla of Vater. A 74-year-old man had undergone a pancreaticoduodenectomy for carcinoma of the papilla of Vater with obstructive jaundice, in April 1995. Histologically, an exposed mass-forming type of tumor, measuring 40 × 30 mm, was composed of mucinous and papillary adenocarcinoma, invading into the muscularis propria of the duodenum, without lymph node metastases. The patient did not receive any type of chemotherapy. In September 1999, a solitary hepatic tumor, 3.5 cm in diameter, was detected in segment VIII of the liver by computed tomography. In November 1999, right hepatic lobectomy was carried out. The anterior and posterior portal pedicles were ligated and dissected in the hepatic parenchyma so as not to compromise the hepaticojejunostomy. After the hepatectomy, the patient was treated with low-dose tegafur/uracil/cisplatin therapy for approximately 2 years. He has been doing well, without recurrence, for 5 years after the hepatectomy. Hepatectomy for hepatic metastases from carcinoma of the papilla of Vater is thought to be a useful surgical treatment in selected patients.     

Nodal involvement as an indicator of postoperative liver metastasis in carcinoma of the papilla of Vater

BACKGROUND/PURPOSE: Although lymph node metastatic involvement is one of the most important prognostic factors for carcinoma of the papilla of Vater, a detailed analysis of this factor in relation to prognosis has not been conducted. METHODS: From 1985 to 2003, 29 patients with carcinoma of the papilla of Vater underwent pancreaticoduodenectomy and dissection of regional lymph nodes at Yamagata University Hospital. We analyzed clinicopathologic variables in relation to prognosis and precisely evaluated nodal involvement in each patient to determine lymphatic flow. Furthermore, the relationship between recurrent site and nodal involvement was investigated. RESULTS: The overall survival rate was 55% at 5 years. The significant prognostic factors were morphological ulcer formation (P = 0.04), histological type (P = 0.03), nodal involvement (P = 0.002), and lymphatic invasion (P = 0.03). Multivariate analysis indicated no independent factor, but nodal involvement may be the strongest prognostic factor. The overall rate of nodal involvement was 41.4% (12 of 29 patients). The metastatic rates in the superior posterior pancreaticoduodenal lymph nodes, the inferior posterior pancreaticoduodenal lymph nodes, the superior mesenteric lymph nodes, and paraaortic lymph nodes were high (31.0%, 20.7%, 17.2%, and 13.8%, respectively). Patients with nodal involvement had a significantly higher rate of liver metastasis after surgery than those without it (P = 0.02). Ulcer formation and histological type were significantly correlated with nodal involvement (P = 0.05 and P = 0.002, respectively). CONCLUSIONS: Nodal involvement is the most important prognostic factor in patients with carcinoma of the papilla of Vater. Patients with nodal involvement are at high risk of liver metastasis; therefore, adjuvant therapy may be necessary for the control of liver metastasis. Preoperative ulcer formation and histological type in the biopsy specimen are good indicators for extended lymph node dissection and adjuvant therapy, because these variables are correlated with nodal involvement. However, our data revealed only the sites of the positive nodes, without addressing the effect of extended lymph node dissection and adjuvant chemotherapy. To date, there has been reporting of extended lymph node dissection and adjuvant chemotherapy in patients with carcinoma of the papilla of Vater. Further studies will be necessary to resolve these problems.     

Endoscopic fistulotomy and biliary drainage as the ultimate palliative treatment for obstruction due to malignant tumors of the papilla of Vater

Summary This report describes the technique and results of endoscopic fistulotomy as a drainage procedure in cases of malignant obstruction of the biliary system from bulky and friable growths in the papilla of Vater. Fistulotomy, coupled with insertion of stents, was successful in seven of eight patients and was associated with relief of jaundice. It is suggested that the method be applied when conventional transpapillary insertion of stents is impossible either due to distortion of the papilla or bleeding of the growth on touch.     

Carcinoma in adenoma of the papilla of Vater

Carcinoma in adenoma of the papilla of Vater is extremely rare. We now report a case of adenocarcinoma in adenoma of the papilla of Vater and the clinicopathological findings are discussed. A 73-year-old Japanese woman was endoscopically diagnosed as a case of carcinoma combined with adenoma of the papilla of Vater. She underwent pancreatoduodenectomy and the postoperative course was statisfactory. Histological examination of the resected tissues revealed a superficial adenocarcinoma in the adenoma of the papilla of Vater. In certain cases, carcinoma of the papilla of Vater may develop from a pre-existing adenoma in the region. Therefore, we recommend that pancreatoduodenectomy should be done when an adenoma presents in this region.     

Ampullectomy for adenoma of the papilla and ampulla of Vater

Introduction: The frequency of malignant adenomas of the papilla figures between 15 and 30%. Villous adenoma is considered to be a premalignant lesion. Treatment: Resection of the papilla is indicated in large tubular and small tubulovillous adenoma. Ampullectomy, however, is mandatory in villous adenoma with severe dysplasia and large villous or tubulovillous adenoma. If villous adenoma with a low-risk pT1 N0 M0 G1/2-cancer is treated by ampullectomy, local lymph dissection should also be performed. Ampullectomy includes extirpation of the ampulla of Vater and reinsertion of the common bile duct and the pancreatic main duct into the duodenal wall. Results: Hospital mortality after ampullectomy is less than 0.4%, and surgical morbidity, e.g., cholangitis, below 10%. Received: 2 March 1998     

Neoplastic diseases of the papilla of Vater

Although it is quite small, the papilla of Vater is an important part of the body. Carcinoma of the papilla may be one of the smallest cancers that can cause death. The 5-year survival rate after resection was 51%, which is not satisfactory. In this article, the topics discussed are (1) pathogenesis, (2) histological characteristics, and (3) the molecular biological characteristics of carcinoma of the papilla of Vater. From results obtained by the investigation of 576 autopsied and 51 resected cases, atypical epithelium was found most frequently in the common channel, where pancreatic juice and bile mix physiologically. Atypical epithelia may be a precursor of carcinoma of the papilla of Vater. Carcinoma of the papilla of Vater could be classified into two types histologically, an intestinal type and a pancreaticobiliary type. The prognosis of patients with the intestinal type was much better than that of patients with the pancreaticobiliary type. These two types of carcinoma should be treated by different operative procedures or adjuvant therapies. Regarding the molecular biological characteristics of carcinoma of the papilla of Vater; (1) K-ras mutation is mainly associated with the intestinal type, and carcinomas of the intestinal and pancreaticobiliary types may develop via different mechanisms; (2) p53 overexpression may play a role in tumor ulceration; and (3) p21/Waf1 overexpression was significantly correlated with a poor prognosis.     

Villous tumor of the papilla of Vater presenting with hypopotassemia

We report a case of villous tumor of the papilla of Vater associated with hypopotassemia. The patient was a 73-year-old woman who presented with jaundice and fever. She had a history of diabetes mellitus and liver dysfunction. Laboratory studies revealed that levels of total bilirubin, alkaline phosphatase, and C-reactive protein, and the white blood cell count were elevated (suggestive of cholangitis) and that the serum potassium level was markedly reduced, to 1.9 mEq/l (normal value 3.5–5.0 mEq/l). Duodenoscopy showed a villous tumor arising in the papilla of Vater. Percutaneous transhepatic biliary drainage was performed. Approximately 700–1500 ml of bile with viscous mucoid fluid was drained daily. Percutaneous transhepatic cholangioscopy showed a papillary lesion in the distal common bile duct. Biopsied specimens from both percutaneous transhepatic cholangioscopy and duodenoscopy disclosed tubulovillous adenoma. Endoscopic ultrasonography showed that the tumor had spread to the main pancreatic duct as well as to the common bile duct. The patient underwent pylorus-preserving pancreaticoduodenectomy. Pathology examination disclosed well differentiated adenocarcinoma, carcinoma in situ, in tubulovillous adenoma. The cancer cells were observed at the bottom of the tumor spreading in the common bile duct. This is a rare case of a patient presenting with hypopotassemia associated with a tubulovillous tumor of the papilla of Vater that secreted mucoid material.     

Vater壶腹及乳头癌的淋巴结转移特点及相关因素

目的 探讨Vater壶腹及乳头癌的淋巴结转移特点及相关影响因素.方法 回顾性分析152例Vater壶腹及乳头癌患者根治术后的淋巴结转移特点,分析其临床病理相关影响因素.结果 对152例Vater壶腹及乳头癌行根治性胰十二指肠切除术,淋巴结转移率为31%,其中T1、T2、T3、T4期淋巴结转移率分别为0、20%（11/54）、26%（19/72）及94%（16/17）.淋巴结转移相关因素有：肿瘤直径（＜3 cm与≥3 cm相比,P=0.002）、肿瘤分化程度（P=0.012）、十二指肠壁浸润（P=0.008）、T分期（P=0.000）及胰腺受侵（P=0.005）.结论 Vater壶腹及乳头癌淋巴结转移率高;根治性手术切除是主要的治疗方式;行肿瘤局部切除时应选择适合的病例.     

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.     

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)     

胰十二指肠切除术治疗胰头和壶腹部癌230例分析

了解胰十二指肠切除术治疗胰头和壶腹部癌的效果和影响病人生存的因素。方法:分析230例胰头和壶腹部癌的主要表现,辅助检查、手术并发症、手术死亡率,调查术后生存情况及影响病人长期生存的因素。结果:本组病人出现黄疽90.9％,腹痛56.1％,发热32.6％,白陶土样大便13.5％。B超、CT和ERCP检查准确率分别为68.3％、79.3％和66.6％。手术并发症72例(31.3％),死亡20例(8.7％)。术后1、3、5年存活率分别为46.7％,17.4％和52％。肿瘤直径、淋巴结转移和切缘状态是影响病人术后生存的因素,而肿瘤分化程度和病人年龄对生存率无明显影响。结论:胰头和壶腹部癌临床表现有多样性,B超和CT等辅助检查有局限性,手术有高并发症,5年生存率低下,手术治疗远近期效果都不理想。     

A long-term surviving patient with cancer of papilla of Vater after local resection

We report successful local resection for cancer of papilla of Vater in an 86-year-old woman. She was referred to our hospital because of right hypochondralgia. Abdominal ultrasonography and computed tomography showed marked dilatation of the common bile duct (CBD). Endoscopic retrograde cholangiography disclosed a small shadow defect in the terminal of the dilated CBD. Biopsy of the papilla revealed well-to-moderately differentiated adenocarcinoma. Considering her extreme old age and keeping in mind her quality of life after the operation, and the finding that the tumor was localized within the papilla and highly differentiated, we performed local resection. In addition, the intrapancreatic portion of the CBD and part of the main pancreatic duct (MPD) were further resected to secure a negative margin, confirmed by frozen section. The MPD was reapproximated to the duodenal mucosa and a choledocho-duodenostomy was performed for CBD reconstruction. Histopathological examination showed the tumor was papillary adenocarcinoma, 10 × 15 mm in size; there was no invasion beyond the sphincter of Oddi, it had partly infiltrated the CBD, but had not invaded to the pancreas or duodenum. The patient's postoperative course was not eventful and she has had good quality of life for the past 6 years since the operation, without any evidence of recurrence. Although radical pancreaticoduodenectomy is now the standard procedure in patients with malignant tumor of the papilla of Vater, local resection is a reasonable alternative for high-risk patients with highly differentiated, apparently localized carcinomas. Received for publication on Aug. 29, 1997; accepted on Jan. 23, 1998     

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.     

扩大Vater壶腹部切除的根治术治疗壶腹部肿瘤

目的 探讨Vater壶腹部肿瘤行壶腹部扩大切除的根治性手术的方法,以减少非肿瘤器官切除,并符合肿瘤治疗原则。方法 总结1995-1998年手术切除的根治术的经验。经十二指肠后外侧入路,整块切除肝外胆道、胆胰管汇合部,壶腹部及十二指肠乳头,十二指肠乳头封闭术。胆、胰、十二指肠间置空肠或胆、胰空肠Roux-en-Y吻合术。结果 围手术期死亡1例。并发症1例经再手术治愈。随访术后半年以上病人9例,最长生存29个月,无并发症及转移征象。结论（1）该术式符合胰十二指肠解剖关系;（2）按肿瘤治疗原则能达到广泛程度清扫;（3）初行该术式者应掌握胰十二指肠切除术。     

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.     

Nodal Microinvolvement in Patients with Carcinoma of the Papilla of Vater Receiving No Adjuvant Chemotherapy

Background  To assess the prognostic significance of nodal microinvolvement in patients with carcinoma of the papilla of Vater. Methods  From 1993 to 2003 at the University Clinic Hamburg, 777 patients were operated upon pancreatic and periampullary carcinomas. The vast majority of patients were operated upon pancreatic ductal adenocarcinoma (n = 566, 73%), followed by carcinomas of the papilla of Vater (n = 112, 14%), pancreatic neuroendocrine carcinomas (n = 39, 5%), intraductal papillary mucinous neoplasms (n = 33, 4%), and distal bile duct carcinomas (n = 27, 3%). Fresh-frozen tissue sections from 169 lymph nodes (LNs) classified as tumor free by routine histopathology from 57 patients with R0 resected carcinoma of the papilla of Vater who had been spared from adjuvant chemotherapy were immunohistochemically (IHC) examined, using a sensitive IHC assay with the anti-epithelial monoclonal antibody Ber-EP4 for tumor cell detection. With regard to histopathology, 39 (63%) of the patients were staged as pT1/pT2, 21 (37%) as pT3/pT4, 30 (53%) as pN0, while 38 (67%) as G1/G2. Results  Of the 169 “tumor-free” LNs, 91 LNs (53.8%) contained Ber-EP4-positive tumor cells. These 91 LNs were from 40 (70%) patients. The mean overall survival in patients without nodal microinvolvement of 35.8 months (median—not yet reached) was significantly longer than that in patients with nodal microinvolvement (mean 16.6; median 13; p = 0.019). Multivariate Cox regression analysis for overall survival revealed that grading was the most significant independent prognostic factor (p = 0.001), followed by nodal microinvolvement (p = 0.013). Conclusions  The influence of occult tumor cell dissemination in LNs of patients with histologically proven carcinoma of the papilla of Vater supports the need for further tumor staging through immunohistochemistry.     

腹腔镜与开腹胰十二指肠切除治疗十二指肠乳头腺癌疗效比较

目的:比较腹腔镜胰十二指肠切除(LPD)与开腹胰十二指肠切除(OPD)治疗十二指肠乳头腺癌的近期及远期疗效。方法:回顾性分析2012年1月—2017年12月共89例行胰十二指肠切除术的十二指肠乳头腺癌患者的临床资料。其中50例行OPD,39例行LPD,比较两组患者的相关临床资料。结果:两组患者一般资料具有可比性。两组手术时间与术中输血例数无明显差异(均P0.05),但LPD组术中出血量明显少于OPD组(P0.05)。LPD组术后住院时间明显短于OPD组(P0.05),但两组术后各并发症发生率、二次手术率、术后30 d死亡方面均无明显差异(均P0.05)。两组R_0切除率及其他术后病理结果均无明显差异(均P0.05)。两组1、3年总生存率与无病生存率均无明显差异(均P0.05)。结论:与OPD比较,LPD治疗十二指肠乳头腺癌患者具有相同的肿瘤根治性,且不增加并发症的发生率,两者远期疗效也相似。     

Recurrent pancreatitis caused by ampullary carcinoma and minor papilla adenoma in familial polyposis: Report of a case

We report a case of relapsing pancreatitis in familial adenomatous polyposis (FAP) with severe duodenal adenomatosis (Spigelman’s stage IV). A 58-year-old man who had undergone total colectomy for FAP 18 years earlier was hospitalized for carcinoma arising from the residual rectum. He had experienced several episodes of upper abdominal pain and ultrasonography and computed tomography (CT) showed diffuse calcification of the atrophic pancreas, suggestive of chronic pancreatitis. He had severe diabetes mellitus, but had no symptoms of pancreatic exocrine dysfunction. Upper endoscopy showed multiple duodenal adenomas including carcinoma involving the papilla of Vater. To remove these duodenal adenomas and ampullary carcinoma and prevent recurrent pancreatitis, we performed pancreaticoduodenectomy. On pathologic examination, the major duodenal papilla was completely obstructed by the carcinoma, and the minor papilla was also involved by the adenoma. The patient has no evidence of disease and has experienced no pancreatitis in 3 years of follow-up.