Transduodenal excision of giant tumour of the ampulla of Vater: a case report

Carcinoma of the papilla is a rare cancer of the digestive tract; 5% of all gastrointestinal tract malignant neoplasms are periampullary. The authors report and discuss the case of one of their patients aged 79 years suffering from a tumour of the papilla. The case was characterized by the large size of the neoplasm (5.5 cm in diameter) and by the poor clinical conditions of the patient, who was suffering from Parkinson's disease and was at high operative risk. The surgical strategy chosen involved transduodenal excision of the tumour with duodenum-bile duct anastomosis and internal duodenum-Wirsung duct anastomosis. The authors first examine the hypothesis that carcinoma of the papilla may represent the evolution of an adenomatous lesion and then go on to assess the therapeutic strategy adopted in the treatment of these neoplasms: in patients at high operative risk a transduodenal excision of the tumour with duodenum-bile duct anastomosis and internal duodenum-Wirsung duct anastomosis may be a valid alternative to the conventional Whipple procedure.     

Adenocarcinoma of the ampulla of Vater. Diagnosis and treatment.

Fifty-one patients underwent operation for adenocarcinoma of the ampulla of Vater. Seven patients underwent palliative bypass, with an operative mortality of 28.6%; 44 additional patients underwent potentially curative pancreaticoduodenal resection (PDR), with an operative mortality of 15.9%. Postoperative complications occurred in 63% of patients. Postoperative gastrointestinal bleeding was observed in 11 of 44 patients who underwent PDR (25%). Although anastomotic ulcers (AU) were directly implicated in five cases (45%), the 12% incidence of AU-related bleeding among 33 patients who underwent PDR without truncal vagotomy (TV) was not significantly different from the 9% incidence observed in 11 patients who underwent PDR plus TV. However, performance of TV appeared to result in a higher incidence of postoperative pulmonary complications. Five patients who underwent curative resection survived for five years (11%). Only one of seven patients who underwent palliative bypass survived three years (14%), and none survived to five years. Acceptable survival rates following resectional therapy warrant an aggressive approach to this tumor. Further, our experience suggests that TV may increase postoperative patient morbidity without actually providing any protection from anastomotic ulceration.     

Carcinoid of the ampulla of Vater: presentation of a new case

The appendix is the most common location for carcinoid tumors within the gastrointestinal tract. Carcinoid tumors of the ampulla of Vater is an extremely rare entity; only 71 cases of carcinoid of the ampulla of Vater have been reported in the literature to date. The clinical picture is non-specific, and endoscopy is the main diagnostic procedure; but large and deep biopsies are often needed to verify the histological nature of the tumor. Surgery is the treatment of choice. We report a new case of this rare tumor, and a review of the literature concerning the clinical findings, diagnosis, therapeutic challenge and results.     

Muir-Torre syndrome: a case for surveillance of the ampulla of Vater

A 64-year-old man with Muir-Torre syndrome (MTS), a condition characterized by the association of multiple sebaceous tumours and kerato-acanthomas with internal malignancies, developed a carcinoma of the ampulla of Vater. This is the 2nd reported ampullary carcinoma in 205 reported cases of MTS, suggesting a higher prevalence in MTS patients than in the general population. As an ampullary carcinoma is potentially amenable to surgical resection, particularly when detected early, patients with MTS should undergo regular endoscopic surveillance of the ampulla of Vater.     

Carcinoma of the ampulla of Vater: comparative histologic/immunohistochemical classification and follow-up

A broad histomorphologic spectrum of ampullary carcinomas of Vater make a reproducible histologic classification difficult. Using cytokeratin immunohistochemistry, we present a new classification of ampullary carcinomas and analyze their clinical significance. Fifty-five invasive carcinomas of Vater's ampulla were histologically classified into pancreaticobiliary, intestinal, and other types. Serial sections of all carcinoma specimens were additionally stained with antibodies to cytokeratins (CK7, CK20), apomucins (MUC1, MUC2, MUC5AC), CEA, CA19-9, Ki67, and p53. Follow-up of patients from 4 months to 22 years after surgery (mean interval, 51.6 months) was evaluated. Most carcinomas of the ampulla of Vater were of immunohistochemically pancreaticobiliary type (iPT, CK7+, CK20-; 54.5%) or intestinal type (immunohistochemically intestinal type [iIT], CK7-, CK20+; 23.6%). Some carcinomas of immunohistochemically "other" type (iOT both CK7+ and CK20+ or CK7- and CK20-; 21.8%) had precursor lesions of iIT or iPT. Carcinomas positive for MUC2 or CEA were associated with iIT (MUC2, P < 0.001; CEA, P = 0.003), whereas MUC5AC-positive carcinomas were related to iPT (P = 0.005). Our classification based on cytokeratin-immunohistochemistry correlated well with the histologic classification according to published criteria (kappa-coefficient = 0.398; P < 0.001). Furthermore, histologically unusual types could be histogenetically related to pancreaticobiliary duct mucosa or intestinal mucosa. Therefore, all 4 signet-ring cell carcinomas were iIT carcinomas. Thus, cytokeratin immunohistochemistry allows a reproducible, histogenetically based categorization of ampullary carcinomas. However, neither histopathologic nor immunohistochemical subgroups significantly correlated with clinical outcome in our German collective. The overall survival was significantly shorter in males (P = 0.032) and patients with positive nodal stage (N1 < N0; P = 0.0025).     

Neuroendocrine tumours of the ampulla of Vater: clinico-pathological features, surgical approach and assessment of prognosis

Background/aims

Neuroendocrine tumours occur very rarely in the ampulla of Vater and their clinical behaviour is unknown. The aim of this study is to assess the clinico-pathological features, surgical approach and prognosis of these patients.

Methods

Six patients with neuroendocrine tumours of the ampulla of Vater treated with curative intent surgery at a single centre were retrospectively analysed. A univariate analysis of potential prognostic factors was also performed (data provided from the present study and literature review).

Results

Pancreaticoduodenectomy was curative in all the patients. Overall and disease-free survival rates were significantly better for G1/G2 tumours (p?=?0.006 and p?=?0.004, respectively). Although frequent, lymph node metastases did not influenced both overall (p?=?0.760) and disease-free survival rates (p?=?0.745). No significant differences of survival were observed in patients with ENETS stage I/II disease, as compared to ENETS stage III disease (p?=?0.169 and p?=?0.137, respectively). No differences were observed according to UICC staging system (p?=?0.073 and p?=?0.177, respectively). Tumours that are less than 2?cm or limited to the ampulla appear to have a better prognosis.

Conclusion

The WHO 2010 classification appear to accurately predict patient prognosis, while the ENETS or UICC staging systems have a limited value (especially in regard to lymph node metastases). Radical surgery (i.e. pancreaticoduodenectomy with lymphadenectomy) should be the standard approach in most patients with NET of the ampulla of Vater because this procedure removes all the potential tumour-bearing tissue.     

Vater壶腹部异位胰腺1例

患者男,70岁.因进行性无痛性黄疸半月余于2000年11月28日入院.经CT,MRCP,B超检查提示肝内胆管扩张,胆总管内经约2.0cm,提示Vater壶腹新生物.诊断:壶腹癌.经术前准备后于2000年12月4日在全麻下行剖腹探查术,术中发现肝脏肿胀淤胆,胆囊扩张,术中探查发现胆总管下段近胰头处可扪及约1cm×1cm×1cm结节,质硬,可活动,与周围组织无粘连,胆道远端完全梗阻,胆总管下段未探及结石,不能排除壶腹周围肿瘤,行胰十二指肠切除术,手术顺利,术后病理报告为十二指肠粘膜下异位胰腺组织.舷     

Embryonal rhabdomyosarcoma of the ampulla of Vater with long-term survival following pancreaticoduodenectomy

Rhabdomyosarcoma of the biliary tree is a rare cause of biliary tract obstruction in childhood. A 3-year-old child is reported here after presenting with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the ampulla of Vater. He underwent pancreaticoduodenectomy followed by adjuvant chemotherapy and irradiation. He is now well and free of disease 5 years following treatment. This child appears to be the first long-term survivor who has required pancreaticoduodenal resection for this lesion.     

Carcinoma of the ampulla of Vater

Experience in treating tumours of the ampulla of Vater in the North West region of the UK is reviewed. The results of local resection, radical resection and palliative bypass in 61 cases have been compared. Local resection in this series offers a better survival than radical procedures at one, two, three, and five years. The operative mortality for radical procedures was 30 per cent. There were no operative deaths in those patients having a local resection. No patient having palliative bypass surgery survived more than 18 months.     

Reimplantation of the ampulla of Vater

Injury to the common bile duct and the pancreatic duct during duodenal ulcer or tumor surgery is exceedingly rare. In the past 50 years, only eight case reports have dealt with reimplantation of the ampulla of Vater. Reimplantation sites have included the stomach, duodenum, and jejunum. Herein we described a new technique that uses the gallbladder for reimplantation of the ampulla of Vater.     

Safety and long-term efficacy of transduodenal excision for tumors of the ampulla of Vater

BACKGROUND: Ampullary tumors should be resected because of the high incidence of malignancy and the unreliability of preoperative endoscopic diagnosis. Controversy exists about whether to perform a transduodenal excision (TDE) or a pancreatoduodenectomy. This study evaluated the safety and long-term efficacy of TDE. METHODS: The records of 21 patients with a pathologic diagnosis of ampullary adenoma who underwent TDE were reviewed. Demographics, symptoms, pathologic findings, and outcomes were analyzed and long-term follow-up was ascertained. RESULTS: Twenty-one patients (mean age, 61 years) underwent TDE. Final pathology showed adenoma in all patients including 1 (5%) with invasive cancer, 2 (9%) with microinvasive cancer, 6 (28%) with high-grade dysplasia, and 1 (5%) with low-grade dysplasia. The overall survival was 85% (mean follow-up of 38 months). One of 3 late deaths was likely related to disease progression. Sixteen of the 18 remaining patients (89%) had no evidence of tumor recurrence. One benign ampullary recurrence was successfully treated endoscopically. One additional patient developed an ampullary cancer and underwent pancreatoduodenectomy. CONCLUSIONS: TDE of benign ampullary tumors, even those with varying grades of dysplasia, can be performed with acceptable morbidity and low rates of recurrence. Postoperative endoscopic surveillance is mandatory to identify recurrent tumors.     

Vater壶腹癌局部切除术后长期存活相关因素的分析

目的:探讨影响Vater壶腹癌行局部切除术后长期生存的相关因素,从而制订出更具针对性的治疗方案.方法:回顾性分析行局部切除术的21例Vater壶腹癌患者的临床资料,采用Kaplan-Meier法进行生存分析,独立样本t检验、Fisher精确检验.结果:全组无手术死亡,总的5年生存率为34.1%;15例患者术后出现明确的肿瘤复发和转移,其中1O例患者(47.6%)出现局部复发,4例患者(19.0%)出现肝转移,1例患者(4.8%)出现腹壁转移;单因素分析表明,肿瘤直径、肿瘤分化程度是影响Vater壶腹癌患者术后生存时间的相关因素(P=0.014,0.015).结论:Vater壶腹癌行局部切除术后预后良好,肿瘤直径和肿瘤分化程度是影响预后的相关因素,该术式适用于肿瘤直径<2 cm、高分化、T1和T2期患者.     

Gangliocytic paraganglioma of the ampulla of Vater

A case of a bleeding duodenal gangliocytic paraganglioma (GPG) of the Papilla Vateri is reported. The 47 year old patient was treated successfully by partial pancreaticoduodenectomy. Histologically the GPG is characterized by three types of cells: epitheloid cells, ganglion-like cells and spindle cells. So far 81 cases are reported in the literature. Most cases of GPGs are benign, metastasis or recurrence is possible. This case showed an infiltrative growth pattern indicating the true neoplastic nature of this tumor.     

Carcinoma of the ampulla of Vater.

Thirty-five consecutive cases of adenocarcinoma of the ampulla of Vater seen over the past 36 years were reviewed. The introduction of new diagnostic techniques over the course of this study improved the accuracy of preoperative diagnosis but did not lead to earlier diagnosis. The surgical resectability rate was 88%, and 53% of postoperative survivors were free of disease at 5 years. Of the 14 patients with metastases to regional lymph nodes, 27% survived disease-free for 5 years. Surgical mortality was 25% for the entire series but has been reduced to 6.6% over the past decade. Surgical mortality was primarily due to leakage of the pancreaticojejunostomy; the risk of pancreaticojejunostomy leak correlated inversely with the degree of chronic pancreatitis in the pancreatic remnant. In 35% of resected cases, a benign adenomatous component was contained within the cancer of the ampulla of Vater. Cure rates are good for this lesion. The most important factor in maximizing cure rate is careful attention to the technical details of pancreaticojejunostomy in order to minimize surgical mortality. Benign adenomas appear to be a frequent precursor of carcinoma of the ampulla of Vater.     

Multimodal treatment of neuroendocrine tumors of the pancreas and the ampulla of Vater

Neuroendocrine tumors are not seen frequently. They are most commonly located in the small bowel including the vermiform appendix. Neuroendocrine tumors of pancreatic origin are extremely rare. Symptoms caused by excessive hormone production by large liver metastases often lead to their diagnosis. Preoperative diagnostics include analysis of specific hormones in serum and urine, ultrasound, CT and somatostatin receptor scintigraphy. Liver metastases of neuroendocrine tumors of the pancreas are common at the time of diagnosis. Curative resection should be performed whenever possible, although patients often benefit from debulking procedures, too. Liver metastases can be subjected to surgical resection, embolization, regional chemotherapy or local procedures such as alcohol injection or cryoablation. As an exception, liver transplantation can be considered in selected cases where radical surgery for the primary tumor could be performed and extrahepatic metastases are not present. Supplementary or alternative options include octreotide and/or interferon-alpha administration. In this article, we report on 6 patients suffering from primary neuroendocrine tumors of the pancreas or the ampulla of Vater who were treated at our department over the past 5 years. In addition, we discuss our own experience with this rare condition in the light of the recent literature.