Radical Surgical Resection for Carcinoid Tumors of the Ampulla

Ampullary carcinoid tumors are extremely rare. The present study describes the clinicopathological features and outcomes for 10 ampullary carcinoid patients who underwent radical resection from 1998 to 2005. During this study period, 294 patients underwent pancreatoduodenectomy for ampullary neoplasms in our institution. The mean patient age was 58.0 ± 13.4 years, and seven were male. Initial clinical manifestations were jaundice in four patients, nonspecific gastrointestinal symptoms in five, and completely asymptomatic in one. Standard pancreatoduodenectomy was performed in three patients, and pylorus-preserving pancreatoduodenectomy in seven, and there were no major complications. The mean tumor size and volume were 2.1 ± 1.3 cm and 4.1 ± 6.9 ml, respectively. Synaptophysin staining was positive in ten patients and chromogranin staining positive in eight. R0 resection was achieved in all ten patients. Overall and disease-free survival rates were 90 and 80% at 1 year, and 64 and 56% at 3 years, respectively. The liver was the most common site of initial metastasis after curative resection. Univariate analyses revealed that a maximal tumor diameter ≥2 cm and tumor extension beyond the ampulla were risk factors for tumor recurrence. In conclusion, while the majority of ampullary carcinoids are indolent, this tumor is associated with a relatively poor prognosis. We believe that radical resection, with the aim of complete tumor removal and cure, is the treatment of choice.     

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.     

Large Cell Neuroendocrine Carcinoma of the Ampulla of Vater: Report of a Case

Large cell neuroendocrine carcinoma in the ampulla of Vater is rare and very different from the common ampullary adenocarcinoma. A 59-year-old man was admitted with obstructive jaundice. Gastroendoscopy showed a swollen ampulla of Vater and pathological examination of an ampullary biopsy revealed findings of a carcinoid tumor. After endoscopic biliary drainage, he underwent pancreaticoduodenectomy. A diagnosis of large cell neuroendocrine carcinoma was confirmed by immunohistochemical examination of the resected specimen. Despite adjuvant chemotherapy with cyclophosphamide and cisplatin, liver and peritoneal metastasis developed within 5 months and he survived for only 10 months after the operation. Thus, further investigations are needed to find a more effective postoperative adjuvant chemotherapy agent to treat patients with this aggressive tumor.     

Carcinoma of the ampulla of vater

Summary Endoscopically placed biliary endoprostheses were used to treat obstructive jaundice in 71 patients with ampullary carcinoma. Successful placement of an endoprosthesis was achieved in 68 patients (95.8%). Bilirubin declined in 67 patients (98.5%). There was no procedure-related mortality. Twenty-two patients (31%) received further surgical therapy, and 47 received an endoprosthesis as their only therapeutic intervention. In the latter group, bilirubin normalized in 44 of 46 patients surviving longer than 30 days (95.7%). Mean survival was 466 days (median 410, range 23-1515), which compares favorably with surgical palliation. Complications mainly involved clogging of the endoprosthesis, which was easily treated endoscopically and, more significantly, duodenal stenoses secondary to continued tumor growth in almost 25% of the patients. Although endoscopic drainage is a safe and effective method of relieving biliary obstruction in patients with ampullary carcinoma, we feel it should be reserved for poor surgical candidates and for those patients with a limited life expectancy due to metastatic disease.     

Prognostic Relevance of Lymph Node Ratio and Number of Resected Nodes after Curative Resection of Ampulla of Vater Carcinoma

Background  Nodal metastasis is considered a major prognostic factor in patients with ampulla of Vater carcinoma (AVC). No study has investigated the significance of the ratio between metastatic and resected/examined lymph nodes (LNR) in patients with AVC. Methods  Demographic, operative, and pathology data, including number of resected/evaluated nodes and LNR, were collected from patients who underwent pancreaticoduodenectomy with radical intent for invasive AVC from 1990 to 2005. Survival rates and recurrence patterns were evaluated and predictors were identified. Results  In 90 evaluable patients (51 males, 39 females, median age 62.5 years), 5-year disease-specific survival (DSS) was 61%. The median number of resected/evaluated nodes was 16 (range: 5–47); 50% of the patients had nodal metastases. The 5-year DSS according to LNR was 75%, 49%, 38%, and 0% for LNR = 0, LNR >0 and ≤0.2, LNR >0.2, and ≤0.4, and LNR >0.4 (P = 0.002), respectively. The 5-year DSS was 81% in patients with >16 resected/evaluated nodes compared with 45% in those with ≤16 resected/evaluated nodes (P = 0.001). On multivariate analysis LNR and a number of resected/evaluated nodes >16 were significant predictors of survival; a number of resected/evaluated nodes >16 was also the only independent predictor of recurrence. Conclusions  After curative resection for AVC, LNR and a cutoff of 16 resected/evaluated nodes are powerful prognostic factors. LNR might represent a major parameter for patient stratification in adjuvant treatment trials.     

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     

Carcinoma Arising from Villous Adenoma of the Ampullary Bile Duct: Report of a Case

Adenocarcinoma arising from the villous adenoma of the ampullary biliary epithelium is an extremely rare disorder. The preoperative diagnosis and treatment of the disease represent a major difficulty. A 67-year-old woman was admitted to the hospital with a chief complaint of jaundice. Preoperative investigations revealed obstructive type jaundice due to a 2-cm mass at the end of common bile duct. She was operated on and after undergoing a sphincterotomy, small, yellowish, grape-like particles fell down from the ampullar orifice. A frozen-section examination of these particles revealed villous adenoma. Next, a transduodenal resection of ampulla and reconstruction were performed. The frozen-section examination of the resected material also revealed a villous adenoma. The patient was discharged uneventfully. The histological examination revealed a villous adenoma arising from the biliary epithelium and some adenocarcinoma foci. The surgical margins were tumor free. Nevertheless, she developed hepatic metastases 15 months after surgery. This case shows the importance of surgeons to keep in mind the fact that frozen examinations may sometimes miss a malignancy and they therefore cannot be relied upon to rule out malignancy in villous adenoma of the ampullary bile duct. This case was presented as a poster presentation at the 12th World Congress of the International Association of Surgeons and Gastroenterologists, Istanbul, Turkey, October 31–November 4, 2002.     

Comparative Value of Mammography,Fine-Needle Aspiration Biopsy,and Core Biopsy in the Diagnosis of Invasive Lobular Carcinoma

Abstract: Invasive lobular carcinoma of the breast (ILC) is a lesion often cited as being more difficult to diagnose than invasive ductal carcinoma. Our objective was to assess the role of mammography, fine-needle aspiration biopsy (FNAB) and large core needle biopsy (LCNB) in the diagnosis of invasive lobular carcinoma of the breast. We reviewed 1,074 consecutive patients with invasive cancers of the breast, of which 102 (9%) were ILC. All the patients with ILC had mammography, 66 had FNAB, and 15 had LCNB performed. Of the patients with ILC, 95 of 102 (93%) had an abnormal mammogram. Seven patients (7%) had a negative mammogram. The most common radiographic finding in patients with ILC was a spiculated mass (42%). The average size of the cancers detected by mammography was 1.17 cm, with no significant effect of size on the ability to detect cancer based on its mammographic presentation for p > .05. Sixty-six patients had FNAB, with a postive diagnosis of cancer in 27 (41%) and suspicious or atypical aspiration in 20 patients (30%). Nineteen patients (28%) had false-negative aspirates. There was no significant difference in size for patients whose cytology demonstrated malignant, suspicious, atypical, or benign aspirates, F (4,56) = 1.01, p > .05. For the fifteen patients who had LCNB, a definitive diagnosis of ILC was made in 13 cases (87%), with another patient having a diagnosis that was “suspicious” for ILC. One patient had a false-negative LCNB. ILC can be detected by mammography, in most instances. FNAB may fail to diagnose this cancer, particularly if the cancer is of low nuclear grade. The lack of reliability of FNAB for diagnosis of ILC, which may occur in some cases is due to the discontinuous infiltrative pattern and the intrinsic cytologic characteristics of this cancer. LCNB is a complementary diagnostic procedure when the FNAB is nondiagnostic to demonstrate the presence of ILC. In the presence of a dominant mass by palpation and a clinical suspicion of the presence of ILC, a negative mammography or a benign FNA should not preclude a surgical biopsy.     

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     

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.     

Local resection of ampullary tumors

There is no consensus on the appropriateness of local resection for ampullary tumors, because malignant recurrence of what were thought to be benign tumors has been reported. This study examined the role of local resection in the management of ampullary tumors. Thirty patients (mean age 66 years) had transduodenal local resections performed at UCSF-Moffitt Hospital or the San Francisco VA Medical Center (February, 1992 to March, 2004). Mean follow-up time was 5.8 years. Preoperative biopsies (obtained in all patients) showed 18 adenomas, four adenomas with dysplasia, five adenomas with atypia, one adenoma with dysplasia and focal adenocarcinoma, and two tumors seen on endoscopy, whose biopsies showed only duodenal mucosa. In comparison with the final pathology findings, the results of frozen section examinations for malignancy in 20 patients, during the operation, were false-negative in three cases. The final pathologic diagnosis was 23 villous adenomas, six adenocarcinomas, and one paraganglioma. On preoperative biopsies, all patients who had high-grade dysplasia and one of five patients with atypia turned out to have invasive adenocarcinoma when the entire specimen was examined postoperatively. Two (33%) adenocarcinomas recurred at a mean of 4 years; both had negative margins at the initial resection. Among the 23 adenomas, three (13%) recurred (all as adenomas) at a mean of 3.2 years; in only one of these cases was the margin positive at the time of resection. Tumor size did not influence recurrence rate. Ampullary tumors with high-grade dysplasia on preoperative biopsy should be treated by pancreaticoduodenectomy because they usually harbor malignancy. Recurrence is too common and unpredictable after local resection of malignant lesions for this to be considered an acceptable alternative to pancreaticoduodenectomy. Ampullary adenomas can be resected locally with good results, but the recurrence rate was 13%, so endoscopic surveillance is indicated postoperatively. Frozen sections were obtained during the operation, but they were less reliable than expected. No adenomas recurred as carcinomas, suggesting that local resection is appropriate for these tumors in the absence of dysplasia or atypia on preoperative biopsies. Presented at the Forty-Sixth Annual Meeting of The Society for Surgery of the Alimentary Tract, Chicago, Illinois, May 14–18, 2005 (poster presentation).     

壶腹腺癌胰十二指肠切除术后预后相关因素分析

目的:探讨影响壶腹腺癌患者行胰十二指肠切除术预后相关因素。方法:回顾2007年1月—2014年12月期间接受胰十二指肠切除术治疗的壶腹腺癌患者的临床及病理资料,分析患者术后生存的影响因素。结果:全组患者术后1、3、5年生存率分别为87.5%、64.3%、54.5%。单因素分析显示,术前总胆红素、直接胆红素、CEA、CA19-9、CA242、肿瘤分化程度、肿瘤浸润深度、淋巴结转移、肿瘤分期、胰腺侵犯、神经侵犯、术后辅助治疗是影响壶腹腺癌患者术后生存的影响因素(均P0.05)。多因素分析显示,CEA、肿瘤浸润深度是影响壶腹腺癌患者术后生存的独立影响因素(均P0.05)。结论:壶腹腺癌患者行胰十二指肠切除术后的预后与多种因素相关,CEA、肿瘤浸润深度是评价壶腹腺癌患者预后最重要的指标。     

Villous tumor of the duodenum: report of a case and review of the literature

Villous tumors of the duodenum are rare tumors which have been infrequently reported in the literature. Surgical treatment options include wide local excision and radical pancreaticoduodenectomy. A case of duodenal villous adenoma presenting with bilious vomiting is presented here. Received: April 25, 2000 / Accepted: November 20, 2000     

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.     

Flowcharts for the management of biliary tract and ampullary carcinomas

No strategies for the diagnosis and treatment of biliary tract carcinoma have been clearly described. We developed flowcharts for the diagnosis and treatment of biliary tract carcinoma on the basis of the best clinical evidence. Risk factors for bile duct carcinoma are a dilated type of pancreaticobiliary maljunction (PBM) and primary sclerosing cholangitis. A nondilated type of PBM is a risk factor for gallbladder carcinoma. Symptoms that may indicate biliary tract carcinoma are jaundice and pain in the upper right area of the abdomen. The first step of diagnosis is to carry out blood biochemistry tests and ultrasonography (US) of the abdomen. The second step of diagnosis is to find the local extension of the carcinoma by means of computed tomography (CT), magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), percutaneous transhepatic cholangiography (PTC), and endoscopic retrograde cholangiopancreatography (ERCP). Because resection is the only way to completely cure biliary tract carcinoma, the indications for resection are determined first. In patients with resectable disease, the indications for biliary drainage or portal vein embolization (PVE) are checked. In those with nonresectable disease, biliary stenting, chemotherapy, radiotherapy, and/or best supportive care is selected.     

Pancreatic Resection for Metastatic Renal Cell Carcinoma: Presentation,Treatment, and Outcome

Background: The diverse natural history of renal cell carcinoma (RCC) includes metastases to the pancreas, a very unusual site for distant spread of other cancers. Considering the relatively indolent behavior of some cases of metastatic RCC, pancreatic resection is offered to select patients.Methods: We reviewed the records of patients at three affiliated university hospital centers who had prior nephrectomy for RCC and subsequent pancreatic resection of metastases.Results: Fourteen patients—9 women and 5 men with a median age of 63.8 years—underwent a total of 15 pancreatic resections for metastatic RCC. Nine (64%) had solitary metastases. The median interval from nephrectomy to diagnosis of pancreatic metastases was 83 months. The median size of metastases was 4.6 cm. There was one perioperative death. Pancreatic recurrence occurred in five patients (36%), and one patient underwent repeat resection. At a median follow-up of 32 months, seven patients (50%) are alive without evidence of disease, and four patients (28%) are alive with recurrent disease.Conclusions: Resection of pancreatic metastases from RCC is associated with long-term survival and should be considered for patients in whom complete resection is possible.