A case of hepatectomy for liver metastasis after pancreatoduodenectomy for carcinoma of ampulla of Vater]

After curative resection of carcinoma of ampulla of Vater, 5-year survival rate has been reported ranging from 40% to 60%. Two major causes of the treatment failure are local recurrence and liver metastasis. Liver metastases are often multiple and are associated with poor prognosis. There have been few reports on long-term survivors after hepatectomy for metastatic liver tumors from carcinoma of ampulla of Vater. We report a 42 year-old female patient with solitary hepatic metastasis from carcinoma of ampulla of Vater, which was successfully treated by hepatectomy 69 months after curative Whipple's operation. Histologic examination of the resected specimen had revealed stage IB moderately-differentiated, intestinal type adenocarcinoma (T2N0M0). Since neither local recurrence or distant metastasis were detected, the patient underwent liver segmentectomy. Histologic study confirmed the presence of metastatic liver tumor from carcinoma of ampulla of Vater. She is doing well without evidence of recurrence at 20 months after hepatectomy.     

Jaundice caused by a clinically undetectable T-cell lymphoma infiltrating the sphincter of Oddi

Malignant lymphoma rarely presents with jaundice. We describe a patient who had a unique etiology for painless jaundice, dilated ducts, and a normal ampulla of Vater. A Whipple's procedure was performed for the suspicion of pancreatic cancer, and initial pathological review detected only mild focal chronic pancreatitis. Seven months later, the patient developed ascites, retroperitoneal mass, and splenomegaly caused by a T-cell lymphoma. Reevaluation of the Whipple's specimen revealed previously unrecognized microscopic infiltration and fibrosis of the sphincter of Oddi by atypical T-lymphocytes. Obstructive jaundice caused by a clinically undetectable primary duodenal T-cell lymphoma has not been previously reported and is contrasted with other causes of jaundice associated with malignant lymphoma and ampullary lesions.     

Actinomycosis mimicking recurrent carcinoma after Whipple＇s operation

Actinomycosis is a rare, chronic, spreading, suppurative, granulomatous and fibrosing infection. Actinomyces are normal inhabitants of the oral cavity and gastrointestinal tract. They rarely cause disease and are seldom reported as pathogens. Herein, we reported on a 69-year-old male patient who had undergone Whipple's operation due to ampulla Vater carcinoma, and became infected with actinomycosis at the pancreaticojejunostomy, which mimicked a recurrent malignancy. He was treated with radical resection of the mass at the pancreaticojejunostomy and had an uneventful postoperative course.     

Primary acinar cell carcinoma of the ampulla of Vater

Acinar cell carcinoma of the pancreatobiliary system is a relatively rare malignant neoplasm arising usually in the pancreatic parenchyma. We experienced a 68-year-old woman who presented with obstructive jaundice due to an ampullary mass 1.0 cm in diameter, detected by abdominal computed tomography and endoscopic examination. The patient underwent a curative surgical operation, and histopathological examination revealed that the tumor was confined to the ampulla of Vater with no continuity to the pancreatic parenchyma. The tumor cells showed acinar or tubular arrangement with eosinophilic to basophilic granular cytoplasm, findings identical to those of acinar cell carcinoma of the pancreas. Immunohistochemically, the tumor cells were positive for lipase. From these findings, we concluded that the tumor was primary acinar cell carcinoma arising in the ampulla of Vater, probably originating from heterotopic pancreatic tissue. This is the first reported case of primary acinar cell carcinoma in the ampulla of Vater.     

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.     

Recurrent acute pancreatitis caused by ampullary villous adenoma

Neoplastic lesions of the ampulla of Vater are a rare cause of acute and recurrent pancreatitis. We have recently had the privilege of studying a 58-year-old woman who presented with recurrent pancreatitis. At endoscopic retrograde pancreatography, a mucosal abnormality of the ampulla of Vater was noted and documented to be a villous adenoma. It was resected surgically and the patient has been symptom free. This report documents the ninth patient in the English literature with pancreatitis secondary to a benign neoplasm of the ampulla of Vater. Six of the nine patients have been male. They have ranged in age from 42 to 68 years. Approximately 30% of villous adenomas of the duodenum contain foci of invasive or in situ carcinoma. Villous adenomas of the ampulla of Vater require aggressive therapy. Further, it has been noted that a number of patients with small intestinal neoplasms have concomitant large-bowel polyps.     

Cephalic duodenopancreatectomy for endocrine tumor of the ampulla of Vater and of the minor papilla]

OBJECTIVES: Endocrine tumors of the ampulla of Vater and minor papilla are rare. This study describes the mode of presentation and evaluates the correlation between pathological features and prognosis. PATIENTS: Between 1982 and 1998, 6 patients (3 M, 3 F, mean age: 47.6 years, range: 36-58) for whom a diagnosis of endocrine tumor of the ampulla of Vater or minor papilla was made between 1982 and 1998 after histological examination of an operative specimen of pancreaticoduodenectomy. RESULTS: One patient was detected incidentally, two had a Zollinger-Ellison syndrome, two had pain and one had obstructive jaundice with pain. The tumor was located in the ampulla of Vater in 5 cases and at the minor papilla in 1 case. All patients underwent a pancreaticoduodenectomy, with histological examination showing tumor diameter varying from 5 to 40 mm and positive lymph nodes. Five patients had a well differentiated endocrine tumor and one a poorly differentiated tumor. All patients had positive Grimelius staining. The secretory profile analyzed by immunohistochemistry was heterogeneous. Median duration of follow-up was 51 months (range: 6 months-16 years) with all patients currently still alive. The patient with a poorly differentiated tumor had diffuse liver metastases, the others were disease-free. CONCLUSION: This study demonstrates the frequency of metastatic spread to adjacent lymph nodes and the inconsistent secretory profiles of these tumors. Pancreaticoduodenectomy may offer long term disease-free survival in well differentiated tumors, and such histology may be useful in advising on prognosis.     

Histamine-producing neuroendocrine tumor of the ampulla of Vater

Neuroendocrine tumors of the ampulla of Vater are rare (less than 100 cases reported). We report here a new case characterized by histamine secretion, a hitherto unreported feature. Clinical presentation is similar to that of other tumors of the ampulla of Vater. In our observation, the patient had noticed urticaria on the right forearm for several months. Tumor of the ampulla was confirmed by endoscopic ultrasonography, while neuroendocrine characterization was assessed on biopsies after endoscopic sphincterotomy. Histamine concentration in blood was the only elevated neuroendocrine marker and returned to normal after surgical resection. Histology showed a neuroendocrine tumor with extension to lymph nodes. On immunohistochemical analysis, production of histamine was confirmed, and the diagnosis of mastocytoma was eliminated. In view of the literature, neuroendocrine tumors of the ampulla of Vater are associated with a good prognosis (5 year-survival rate: 90%) despite early lymph node involvement.     

Signet ring cell carcinoma of the ampulla of Vater

Introduction

Signet ring cell carcinoma of the ampulla of Vater is a rare entity and less than 20 cases have been described in the literature. We report the cases of two patients with this disease and provide a literature review of previous studies.

Case report

We describe two patients with obstructive jaundice. Abdominal ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic and common bile duct. Duodenoscopy indicated a protruding mass on the ampulla of Vater. Histopathological examination showed round cells and their nuclei were located on one side with prominent signet-ring features. One patient underwent a cephalic pancreatoduodenectomy with lymphadenectomy and the other a total pancreatectomy.

Discussion

Signet ring cell carcinoma of the ampulla of Vater has only been described in isolated cases in the literature. Therefore, the clinicopathological features and prognosis of this disease have not yet been well defined.     

Synchronous primary carcinomas of the ampulla of Vater and ascending colon in a patient with multiple flat adenomas

Multiple primary cancers occurring in the same patients have been reported to represent 1.8–3.9% of all cancers. The majority of all patients reported to have had a combination of simultaneous neoplastic changes in the ampulla of Vater and the colon showed familial adenomatous polyposis (FAP) syndrome. Variants of familial adenomatous polyposis coli are: attenuated adenomatous polyposis coli (AAPC, previously also known as flat adenoma syndrome) and multiple adenoma coli. AAPC is characterized clinically by many, but usually fewer than 100, colonic lesions that are characteristically slightly elevated and plaque-like, with a reddish surface and sometimes central depression. Genetically it represents an extremely rare variant of FAP. Another group of individuals, so-called multiple adenoma patients, have a phenotype similar to AAPC, but most have no demonstrable germ-line adenomatous polyposis coli mutation, as do patients with FAP or AAPC. However, there have been only a few reports that discussed concurrent neoplastic changes in the ampulla of Vater and colon in patients with multiple colonic flat adenomas, but without the florid phenotype of classical FAP. We present rare clinical course of a patient with multiple (more than 60) flat adenomas in the proximal colon and two primary cancers: of the ampulla of Vater and of the ascending colon. This patient and his family history did not show polyposis compatible with FAP or hereditary nonpolyposis colorectal cancer (HNPCC) syndrome.     

Signet-ring-cell carcinoma of the ampulla of Vater: a case report

We encountered a rare variant of carcinoma of the ampulla of Vater in a 68-year-old man with postprandial abdominal pain and nausea. Ultrasonography and computed tomography showed a dilated common bile duct and main pancreatic duct. At duodenoscopy, the papilla of Vater was enlarged, and biopsy specimens taken from the papilla revealed signet-ring-cell carcinoma. Endoscopic ultrasonography showed a hypoechoic tumor without pancreatic invasion. Pylorus-preserving pancreatoduodenectomy was performed. Histologic examination of resected specimens indicated lymphatic and vascular invasion, but surgical margins were tumor-free. The patient is alive and disease-free 10 months after the operation.     

Coexistent Ampullary Squamous Cell Carcinoma with Adenocarcinoma of the Pancreatic Duct

Primary squamous cell carcinoma (SCC) of ampulla has seldom been reported. However, metastatic SCC to ampulla of Vater is well known. We report a case of primary SCC of ampulla of Vater coexistent with well-differentiated adenocarcinoma of the distal pancreatic duct. A 50-year-old female presented with evidence of obstructive jaundice. Endoscopic retrograde cholangio-pancreatography revealed bulging papilla with ulcero-infiltrative growth at the ampulla of Vater. An initial endoscopic biopsy of the ampullary mass showed a well-differentiated SCC. The patient underwent Whipple''s operation. Thorough sampling of the dilated portion of the pancreatic duct showed presence of well-differentiated adenocarcinoma of the distal pancreatic duct. Immunohistochemical study with synaptophysin and chromogranin was done with negative result, ruling out neuroendocrine differentiation. Also, a detailed clinical, endoscopic and radiological examination was carried out, that excluded the presence of primary SCC elsewhere.     

Surgical technique for complete resection of the extrahepatic portion of the common bile duct and the ampulla of Vater for tumors of the ampulla of Vater

Background

Ampullary tumors have to be completely resected, but substantial morbidity and mortality rates are associated with pancreaticoduodenectomy (PD). Local resection can be the procedure of choice in selected ampullary lesions for high-risk patients.

Methods

Preoperative examination indicated that the ampullary tumor extended into the common bile duct without evidence of pancreatic duct involvement and no definite invasion into either the duodenum or the pancreas. We performed a complete resection of the extrahepatic bile duct and the ampulla of Vater, including the tumor, without performing PD by dissecting the intrapancreatic bile duct from the pancreas both downward towards the ampulla of Vater and upward using a transduodenal approach.

Results

The operation was successfully completed, and the postoperative course was uneventful, with the exception of a minor pancreatic fistula from retropancreatic dissection. The final pathological examination demonstrated well-differentiated tubular adenocarcinoma limited to the mucosa with negative surgical margins.

Conclusion

Complete resection of the extrahepatic bile duct and the ampulla of Vater through a transduodenal approach can be a feasible and safe surgical procedure for selected ampullary tumors in high-risk patients.     

Ampullary somatostatinoma in a patient with von Recklinghausen's disease

We report a case of somatostatinoma of the ampulla of Vater associated with von Recklinghausen's disease in a 44-year-old woman. On admission the patient was jaundiced, and percutaneous Cholangiodrainage was performed. Cholangiography revealed stenosis of the common bile duct at the lower end Duodenoscopy showed a yellowish tumor of the ampulla of Vater, and the biopsy specimens showed no malignant cells. Pylorus-preserving pancreaticoduodenectomy was performed. Histologically, the tumor was composed of small round cells with a solid or trabecular pattern and with multiple psammoma bodies. Immunohistochemical examination showed that the tumor cells stained for somatostatin. Genomic examination showed neither K-ras nor p53 gene mutations of the resected specimen.     

Primary large B-cell lymphoma of the ampulla of Vater

Primary lymphoma of the ampulla of Vater is rare. The clinico-pathological and interesting endoscopic and radiological features of a patient with this disorder is presented.     

Upper digestive hemorrhage caused by ulcerated periampullary leiomyoma

Duodenal leiomyoma represents a very unusual cause for acute gastrointestinal bleeding. The authors report the case of a 49-year-old male with a massive bleeding from an ulcerated duodenal leiomyoma involving the ampulla of Vater. An emergency Whipple's pancreaticoduodenectomy was carried out under suspicion of local malignancy. It is emphasized that the proper diagnosis of smooth muscle tumors is often difficult, both clinically and from the pathological point of view.     

Cancer of the ampulla of Vater: results of a 20-year population-based study

BACKGROUND: Relatively little attention has been given to the epidemiology and management of cancer of the ampulla of Vater. SETTING: A series of 111 patients with a cancer of the ampulla of Vater diagnosed over a 20-year period (1976-1995) in a well-defined French population was used to analyse its incidence, management and prognosis as well as to determine time trends. Prognosis was determined by using crude and relative survival rates. Factors predictive of survival were also identified using a relative survival model in a multivariate analysis. RESULTS: Age-standardized incidence rates were 3.8 per 1000000 inhabitants in men and 2.7 per 1000000 inhabitants in women. Incidence increased over time in men from 1.9 during the first period (1976-1980) to 5.9 during the last period (1991-1995). In women, incidence rates remained stable. A resection for cure was performed in 52 cases (48.1%). Overall, 9.9% of these cancers were classified TNM stage I and 54.1% stage IV. There was no significant variation in treatment modalities and in stage at diagnosis over the study period. The overall operative mortality rate was 7.5%. Relative survival rates were 58.9% at 1 year, 30.9% at 3 years and 20.9% at 5 years. Five-year relative survival rates varied from 72.8% in TNM stage I cancers to 6.6% in TNM stage IV cancers. Age, treatment procedure and stage at diagnosis significantly influenced the prognosis of cancer of the ampulla of Vater. In a multivariate analysis, stage at diagnosis remained the major prognostic factor (P<0.01). CONCLUSIONS: Although its incidence is increasing in men, cancer of the ampulla of Vater remains a rare tumour in both sexes. No improvements in the management and care of patients have been achieved. Further studies are needed to enhance the understanding of this cancer.     

Peritoneal recurrence of ampullary carcinoma following curative pancreatoduodenectomy

A 72 year-old Japanese man with peritoneal recurrence of carcinoma of the ampulla of Vater after curative pancreatoduodenectomy is presented. He was treated by percutaneous transhepatic biliary drainage (PTBD) for obstructive jaundice. The PTBD catheter dislodged 14 days later. He underwent emergency open peritoneal lavage and external choledochal drainage for diffuse bile peritonitis. Cytologic examination of bile obtained from the T-tube revealed malignant cells. He underwent pancreatoduodenectomy with regional lymph node dissection 2 months later for ampullary carcinoma. Pathologic examination showed a macroscopic protruding, 8 x 7 x 10 mm, papillary adenocarcinoma of the ampulla of Vater. The tumor was classified as stage II with pT2, pN0, and pM0. Eight months later, cytologic examination of ascites demonstrated adenocarcinoma cells. The patient died with peritoneal recurrence 10 months after curative pancreatoduodenectomy.     

Paraganglioma of the ampulla of Vater: a potentially malignant neoplasm

Paragangliomas are rare tumours originating from neuroectodermic remnants and are usually considered as benign. We present two cases of paraganglioma of the ampulla of Vater that were treated surgically by pancreaticoduodenectomy. In one case, histopathology revealed malignant characteristics of the tumour with invasion of the pancreas and simultaneous duodenal lymph-node metastases. Both patients had a favourable outcome without disease recurrence at 40 and 44 months postoperatively. Only 21 cases of ampullary paraganglioma have been reported in the literature, 7 of them with malignant characteristics. In conclusion, paragangliomas of the ampulla of Vater have malignant potential. Surgical therapy of these tumours should not be limited to local resection, as disease recurrence and lymph node involvement have been reported. We propose that paragangliomas of the ampulla of Vater should be operated by cephalic pancreaticoduodenectomy, which allows long-term and disease-free survival.     

Primary duodenal diffuse large cell non-hodgkin lymphoma with involvement of ampulla of Vater: Report of 3 cases

Primary gastrointestinal system lymphomas constitute about one third of all extranodal lymphomas. Duodenal involvement of the lymphoma is a rare condition. Periampullary lymphoma or lymphomatous involvement of ampulla of Vater is even rarer. Since, periampullary lymphoma is not easy to differentiate from epithelial carcinoma of these sites clinically and radiologically, accurate histopathological diagnosis is essential to plan optimal treatment strategy. Obstructive jaundice and the need for some form of drainage procedure and dose modification of the chemotherapy are additional challenges. In this report, we present three cases of duodenal diffuse large cell lymphoma with involvement of ampulla of Vater, two of whom presented with the initial signs of obstructive jaundice. One of the icteric patients was only diagnosed histopathologically following an explorative laparotomy with the initial diagnosis of carcinoma.