Successful Resection of Ampullary Carcinoma in a Father and Adenoma in a Daughter with Familial Adenomatous Polyposis Following Detection by Surveillance: Report of Two Cases

This report describes the successful resection of ampullary carcinoma in a 58-year-old man and an adenoma in his 28-year-old daughter after they had undergone proctocolectomy for familial adenomatous polyposis (FAP). Both patients had been monitored by surveillance endoscopy once a year since their proctocolectomy. The father was found to have an ampullary adenoma 26 years after proctocolectomy, and ampullary carcinoma was detected 2 years later, for which pancreatoduodenectomy was performed. Histological examination of the specimen revealed an ampullary carcinoma, 2.5 × 1.7 cm, that had invaded the submucosal layer, but no lymph node metastasis was found. The patient's daughter underwent endoscopy, which showed an ampullary polyp 6 years after total colectomy. Endoscopic mucosal resection of the peri-ampullary lesion was performed, and histological examination revealed a dysplastic tubular adenoma 0.6 × 0.4 cm in diameter. This report reinforces the importance of long-term periodic surveillance of patients with FAP by gastroduodenal endoscopy. Received: January 9, 2001 / Accepted: July 17, 2001     

壶腹部癌的诊断和治疗

壶腹部癌的发病率高于胆管下端癌，但远低于胰头癌。据统计，其发病率仅为胰头癌的1／12。近年，随着消化内镜的普及，壶腹部癌的发现率逐渐增加。与胆管下端癌及胰头癌比较，壶腹部癌的预后相对较好，手术切除率高，术后5年生存率为30％-70％，诊断和治疗有其独自的特点。     

壶腹部癌的诊断和治疗

Ampullary cancer is a relatively uncommon cancer,which is often considered to have a best prognosis among periampullary cancers.Preoperative endoscopic uhrasonography and transpapillary intraductal ultrasonography Call provide useful information not only for tumor staging but also for making therapeutic decisions,especially in patients who are appropriate for endoscopic papillectomy.Whipple resection and pylrus preserring panereaticoduodenectomy are considered to be the standard treatment for ampullary cancer.Although transduedenal ampullectomy is regarded as a less-invasive treatment compared with Whipple resection,it has a high morbidity and hish rate of cancer-cell remnant at the resected margin.Endoscopic papiilectomy may be the treatment of choice for selected cases of ampullary cancer. As to unresectable ampullary cancer,the performance of a biliary-enteric bypass is considered routine to solve obstructive ianndice.The decision as to whether to perform gastrojejunostomy in patients without obvious gastroduodenal obstruction secondary to the tumor remains controversial.We believe that prophylactic gastrojejunostomy should be performed routinely when a patient is undergoing surgical palliation for unresectable ampullary cancer.     

早期壶腹癌的外科手术进展

十二指肠乳头及壶腹癌是一种较少见的恶性肿瘤.目前主要以手术治疗为主,以胰十二指肠切除术为主要术式.对于早期壶腹癌的治疗有学者提出以局部切除或内镜下乳头切除.局部切除及内镜下切除具有手术死亡率、术后并发症发生率低等优势.但由于缺乏大宗的病例对照研究,对于早期壶腹癌的局部切除治疗的效果仍存在争论.     

Is Blind Pancreaticoduodenectomy Justified for Patients with Ampullary Neoplasms?

Background  Many specialists justify pancreaticoduodenectomy (PD) for pancreatic head neoplasms with suspected but unproven malignance (blind-PD). Our aim in this study was to determine whether blind-PD is also justified for ampullary neoplasms. Methods  We retrospectively reviewed the records of all patients with presumed resectable ampullary neoplasms treated at the National Taiwan University Hospital from 1998 to 2008. Results  Of the 84 patients without a preoperative tissue diagnosis of malignance, 64 had blind-PD and 20 had ampullectomy (AMP) with intraoperative frozen section. Patients with jaundice, gastrointestinal bleeding, imaging findings showing tumor invasion, and larger tumor size were significantly more frequently treated by blind-PD. Final pathological diagnosis was benign in ten of 64 blind-PD-treated patients. Conclusions  Our data support a selective use of blind-PD because (1) a significant portion (65%) of benign ampullary neoplasms can be safely and effectively treated by AMP, (2) blind-PD does not treat ampullary cancer at earlier stage, and (3) blind-PD is associated with significantly more complications and significantly longer hospital stay than AMP. However, blind-PD is strongly recommended for patients with large ampullary neoplasms (>3 cm in diameter), with jaundice, or with malignant endoscopic appearance.     

壶腹癌的外科治疗与预后分析

目的：评价不同手术方式对壶腹癌的治疗效果,并分析其与预后的关系。方法：回顾性研究81例壶腹癌患者的临床资料,根据治疗方法不同,50例采用肿瘤切除术治疗,31例采用姑息性手术治疗。分析不同治疗方法的术后并发症发生情况及预后。结果：肿瘤切除患者的术后并发症发生率差异有统计学意义(P＜0.05),肿瘤局部切除患者的术后并发症发生率比行胰十二指肠切除术低。行不同方法的姑息性手术患者,术后并发症的发生率差异无统计学意义(P＞0.05)。采用肿瘤切除治疗后,患者的生存时间差异无统计学意义(P=0.475),但采用不同方法的姑息性术式治疗后,患者的生存时间差异有统计学意义(P＜0.001)。研究发现,壶腹癌行肿瘤切除术后的中位生存时间比行姑息性手术长。姑息性手术方式中,行ERBD术后的中位生存时间比胆肠吻合和经皮经肝胆道穿刺引流治疗的长。结论：壶腹癌行根治性胰十二指肠切除术后并发症发生率相对较高,但术后生存时间长。     

Ampullary carcinoid tumors: Rationale for an aggressive surgical approach

Two cases of ampullary carcinoid tumor are reported. These tumors are among the most rare of GI tract carcinoids and appear to have a distinct presentation and biological behavior from carcinoids arising in the duodenum. The existing literature is reviewed with attention to the implications for surgical management of this rare disease.     

A case of severe necrotising pancreatitis following ampullary biopsy

We present a case of necrotising pancreatitis following ampullary biopsy in a patient with Barrett’s oesophagus. The patient needed multiple necrosectomies and several admissions to the intensive care unit. This report is only the third and most severe case of pancreatitis following ampullary biopsy, highlighting its importance as a complication.     

Open Sphincteroplasty for Benign Ampullary Stricture After Pancreas Transplantation

Ampullary and proximal pancreatic duct strictures are well known to result in recurrent episodes of pancreatitis in the native pancreas, which when benign in origin can often be treated with sphincteroplasty (open or endoscopic) and stenting in the native pancreas. However, recurrent episodes of pancreatitis in a transplanted pancreas allograft can have multiple potential etiologies, and if the diagnosis of pancreatic duct stricture is made, treatment with preservation of the pancreatic allograft can be challenging. This is the first case report to describe the open sphincteroplasty of a short benign ampullary stricture in a transplant pancreas allograft.     

Endocrine cells in ampullary carcinoma

Background/Purpose  As ampullary carcinoma originates from four anatomical regions, it may have different behaviors depending on its origin. We documented the presence of endocrine cells found in ampullary carcinoma, and we studied the clinicopathological implications of their presence. Methods  We immunohistochemically examined the presence of an endocrine component in 62 surgically resected specimens of ampullary carcinoma, and we studied the clinicopathological differences between endocrine component-positive cases and endocrine component-negative cases. Results  Endocrine cells were detected in 16 cases (26%); 11 cases had many endocrine cells, and five cases had scattered endocrine cells. Serotonin-positive cells were detected in all 16 cases, in which six cases had many positive cells. Several somatostatin-positive cells were detected in three cases. Endocrine cells were detected in ampulloduodenal polypoid lesions (two cases) and ampullopancreaticobiliary ducts (14 cases). The histology of 15 of the 16 endocrine component-positive ampullary carcinomas was the intestinal type. Pancreatic invasion and lymph node involvement were observed less frequently in endocrine component-positive cases (P < 0.01). There were no significant differences with respect to immunoreactivity for carbohydrate antigen (CA) 19.9, carcinembryonic antigen (CEA), and p53 overexpression, and K-ras mutations. Conclusions  Endocrine component-positive ampullary carcinoma seemed to be derived from the ampullopancreaticobiliary common duct or the ampulloduodenum, and to behave less aggressively than endocrine component-negative carcinoma.     

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.     

Periampullary choledochoduodenal fistula in ampullary carcinoma

Most patients with ampullary carcinoma have obstructive jaundice without cholangitis. We experienced a patient with ampullary carcinoma who presented with obstructive jaundice and cholangitis, probably because of an accompanying periampullary choledochoduodenal fistula. A 77-year-old Japanese man had jaundice, high fever, and upper abdominal pain and was diagnosed, at another hospital, with obstructive cholangitis. On admission to our hospital, his symptoms and signs had subsided spontaneously. Abdominal ultrasonography showed cholecystolithiasis and dilatation of the common bile duct. Duodenoscopy showed an ulcerating tumor at the oral prominence of the ampulla of Vater and a periampullary choledochoduodenal fistula at the bottom of the ulcer. Biopsy from the fistula showed well differentiated adenocarcinoma. With a diagnosis of ampullary carcinoma with fistula formation, the patient underwent pylorus-preserving pancreatoduodenectomy. The diagnosis was confirmed by histology. This communication presents a unique case of ampullary carcinoma that caused obstructive jaundice, which subsided spontaneously but was associated with cholangitis caused by the divergent effects of the periampullary choledochoduodenal fistula formed by the carcinoma. Received: September 5, 2000 / Accepted: December 22, 2000     

局部切除与胰十二指肠切除治疗Vater壶腹癌的临床对比研究

目的 为治疗Vater壶腹癌提供一种简单、实用的新方法。方法 应用局部切除与胰十二指肠切除两种不同的方法治疗Vater壶腹癌,判定Vater壶腹癌局部切除的可靠性。结果 Vater壶腹癌局部切除组21例,平均住院14d。发生手术并发症1例,无围手术期死亡,1年、3年、5年生存率分别为95％、69.7％、38.7%。胰十二指肠切除组16例,平均住院21d,发生手术并发症7例,围手术期死亡2例,1年、3年、5年生存率分别为86.7%、68.9%、36.8%。手术并发症两种方法有非常显著差别（P＜0.01）,围手术期死亡及1年、3年、5年生存率无显著差别（P＞0.05）。结论 Vater壶腹癌局部切除是一种合理治疗Vater壶腹癌的手术方法。     

FAP related periampullary adenocarcinoma

INTRODUCTIONThe risk of periampullary neoplasia in patients with familial adenomatous polyposis (FAP) is significantly increased compared to the general population.PRESENTATION OF CASEWe herein report the case of a 47-year-old woman with classic familial adenomatous polyposis with a history of total proctocolectomy for FAP who presented with an ulcerous ampullary lesion 8 years after primary colorectal surgery. Interestingly, the patient had not enrolled to optimal postoperative upper endoscopy follow-up. The patient underwent a Whipple procedure. Histology demonstrated a T2N0 ampullary adenocarcinoma.DISCUSSIONPeriampullary disease in patients with familial adenomatous polyposis occurs increasingly, especially in the subset of patients without proper endoscopic follow-up. Current recommendations concerning upper endoscopy and appropriate management are herein discussed; the importance of optimal postoperative endoscopy after total proctocolectomy in the FAP setting is discussed.CONCLUSIONPeriampullary cancer carries a significant risk in patients with FAP and proper endoscopic follow-up should be applied in this special patient group in order to manage ampullary manifestations of the disease in a timely manner.     

Prognosis of Resected Ampullary Adenocarcinoma by Preoperative Serum CA19-9 Levels and Platelet-Lymphocyte Ratio

Background  The objective of this study was to evaluate whether preoperative CA19-9 levels and the platelet–lymphocyte ratio (PLR) might reflect prognostic indices for resected ampullary adenocarcinoma. Materials and Methods  Data were collected prospectively over a 10-year period for consecutive patients undergoing pancreatoduodenectomy for malignancy. Results  Both preoperative PLR and CA19-9 results were available in 52 cases of resected ampullary adenocarcinoma. Preoperative CA19-9 levels of ≤150 kU/l (or ≤300 kU/l in the presence of bilirubin levels >35 μmol/l) and a PLR of ≤160 were found to represent the optimal cut-off values to risk stratify patients. If both levels were elevated (n = 8), patients had a median overall survival of 10.1 months. If either CA19-9 or PLR were elevated individually (n = 23), patients had a median survival of 25.2 months. For cases where both levels were less than the cut-off values (n = 21), the median overall survival time was not reached but was greater than 60 months (log rank, p < 0.001). This preoperative risk stratification was found to remain a significant independent predictor of survival on multivariate analysis (Cox, p = 0.001) alongside resection margin status (p = 0.002) and tumor size (p = 0.051). Conclusions  Preoperative CA19-9 and PLR both merit further evaluation as prognostic indices in resected ampullary adenocarcinoma.     

胰头癌与壶腹癌可切除性及临床行为特点差异的比较研究

目的探讨胰头癌与壶腹癌在可切除性、肿瘤局部浸润、血管侵犯、淋巴结转移、远处转移、预后等临床行为特点的差异。方法同期收治的胰头癌42例、壶腹癌26例为本研究对象，以手术发现和病理诊断作为金标准，探讨上述指标两组之间的差异。结果同期收治的壶腹癌26例和胰头癌42例中，壶腹癌切除率84．62％，胰头癌的手术切除率为19．05％，胰头癌发生血管侵犯为83．33％，壶腹癌为11．54％，两组间均存在显著差异（P〈0.01）。在肿瘤局部组织浸润（P=0.13）、淋巴结转移（P=0．15）、远处转移（P=0.54），两组间无差异；两组TNM分期构成亦存在明显差异（P〈0．01）。结论壶腹癌与胰头癌因其原发组织、生长部位不同，其临床行为特点各异，术前应力争明确肿瘤部位以利于制定正确治疗方案。     

壶腹部癌的临床病理特征及诊断与治疗

目的 总结壶腹部癌的临床病理特征,探讨该病的诊断及治疗方法.方法 回顾性分析2000年1月至2010年12月北京协和医院收治的187例壶腹部癌患者的临床资料,根据手术方式将患者分为胰十二指肠切除术组(162例)和局部切除术组(25例),观察壶腹部癌的临床病理特征,探讨该病的诊断和治疗方法,分析两组患者的治疗效果.计量资料采用t检验,计数资料采用x2检验,Kaplan-Meier法绘制生存曲线,生存率比较采用Log-rank检验.结果 本组患者行B超、CT、MRI、ERCP检查阳性率分别为9.3％(15/161)、43.9％ (65/148)、21.3％ (19/89)、83.9％( 135/161).高分化腺癌87例,中分化腺癌64例,低分化腺癌27例,腺管癌变9例.T1、T2期壶腹部癌患者行胰十二指肠切除术与局部切除术的生存率比较,差异无统计学意义(x2 =3.163,P＞0.05);T3、T4期壶腹部癌患者行胰十二指肠切除术的预后优于行局部切除术者(x2=6.309,P＜0.05).结论 壶腹部癌以高分化腺癌为主.影像学检查中ERCP确诊率最高.T1、T2期壶腹部癌患者行局部切除术已达到根治目的,而T3、T4期患者应行胰十二指肠切除术.