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.     

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.     

Gastrointestinal stromal tumor of the ampulla of Vater:A case report

Gastrointestinal stromal tumors(GISTs)usually develop in the stomach and small intestine and only rarely occur at the ampulla of Vater,with only 11 cases reported in the literature.We report a case of a GIST of the ampulla of Vater.A 36-year-old,previously healthy man presented with a loss of consciousness lasting a few minutes.A gastroduodenal endoscopy revealed a submucosal tumor with central ulceration at the ampulla of Vater.The enhanced computed tomography scan revealed a smooth-outlined hypervascular solid mass(24 mm×30 mm)in the second part of the duodenum.Neither lymphadenopathy nor metastasis was observed.Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed normal bile and pancreatic ducts.Biopsies were collected from the ulcerative lesion,and the tumor was diagnosed as a GIST.A submucosal tumor with central ulceration may be a characteristic form of GISTs of the ampulla of Vater,and biopsy studies are useful for the diagnosing such tumors.The patient underwent pancreatoduodenectomy,and the operative specimen revealed a 2.2-cm GIST with 1 mitosis per 50 highpower fields.The gold standard for treatment of GISTs is surgical resection without rupture of a capsule.If technically possible,local resection may be considered.However,when the location of the lesion presents challenges,a pancreatoduodenectomy should be performed for GIST of the ampulla of Vater.     

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.     

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.     

Large cell neuroendocrine carcinoma of the ampulla of Vater

BACKGROUND: Neuroendocrine tumors of the ampulla of Vater are extremely rare, and few cases of large cell neuroendocrine carcinoma (LCNEC) of the ampulla have been reported. METHODS: A 48-year-old male with obstructive jaundice was admitted to our hospital. On examination the patient was found to have a periampullary growth and subsequently underwent the Whipple's procedure. RESULTS: Histopathological examination and immunohistochemistry revealed features of LCNEC of the ampulla of Vater. The patient developed multiple liver metastases 6 months after Whipple's procedure. CONCLUSION: LCNEC of the ampulla of Vater is rare and highly aggressive, with a dismal prognosis.     

Small cell neuroendocrine carcinoma of the ampulla of Vater with foci of squamous differentiation: a case report

A 54-year-old woman with obstructive jaundice was found to have a 4-cm ulcerated, elevated tumor, located at the papilla of Vater by endoscopy and radiographic investigation. Based on a clinical diagnosis of carcinoma of the ampulla of Vater, a pylorus-preserving pancreaticoduodenectomy was performed. The histologic appearance of the lesion was identical to extrapulmonary small cell carcinoma, with diffuse proliferation of small, spindle-shaped, atypical tumor cells with numerous mitoses. Neuroendocrine differentiation was demonstrated by immunoreactivity with neuron-specific enolase (NSE) and Leu-7, and by the presence of dense core granules ultrastructurally. The tumor was composed mainly of small cell neuroendocrine carcinoma, and partially of an area of squamous differentiation, showing transition from one to the other. Histopathologic investigation disclosed direct invasion deep to the lamina propria, and infiltration of the pancreatic parenchyma, duodenum, and bile duct. Lymph node metastases were present in the superior pancreaticoduodenal chain. The patient died of liver metastases 8 months later. As in previously reported cases of small cell neuroendocrine carcinoma of the ampullary region, the present case showed extremely aggressive clinical behavior with early metastases resulting in fatal outcome.     

Undifferentiated carcinoma of the duodenal ampulla

This report demonstrates a case of undifferentiated carcinoma of the duodenal ampulla. A 74-year male experienced jaundice lasting for 3 weeks. An upper gastrointestinal series demonstrated a polypoid, ovoid filling defect in the second portion of the duodenum, and duodenoscopy disclosed a protruding mass involving the orifice of the papilla of Vater. Cholangiography demonstrated obstruction due to compression in the terminal common bile duct. Pylorus-preserving pancreatoduodenectomy was performed on the diagnosis of ampullary carcinoma. The gross specimen showed a polypoid mass, measuring 3.5 cm in diameter, in the ampulla, located mainly in the duodenal submucosal layer and invading the terminal common bile duct. Histologically, the tumor was small cell type, undifferentiated carcinoma, arising from the duodenal epithelium adjacent to the ampulla.     

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.     

Coexistence of small cell neuroendocrine carcinoma and villous adenoma in the ampulla of Vater

Small cell neuroendocrine carcinoma of the ampulla of Vater is extremely rare and different from the common ampullary adenocarcinoma. The ampullary adenoma is also a rare neoplasm and has the potential to develop an adenocarcinoma. Their coexistence has been rarely reported in the literature. We herein describe an unusual case of a small cell neuroendocrine carcinoma associated with a villous adenoma in the ampulla of Vater with emphasis on computed tomography （CT） and histopathological findings. We also discuss their clinical, histopathological and radiological features as well as possible histogenesis.     

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.     

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.     

Synchronous double primary cancer of the esophagus and ampulla of Vater successfully treated by a two-stage operation: report of a case

A 68-year-old man was diagnosed with local recurrent cancer of the ampulla of Vater by follow-up endoscopy 3 years after an endoscopic papillectomy. A screening endoscopy found superficial middle thoracic esophageal cancer. The patient required an esophagectomy and pancreatoduodenectomy. We chose a two-stage operation for the esophageal cancer and the local recurrent cancer of the ampulla of Vater, both to reduce surgical invasiveness and to circumvent the lower curability. The first-stage operation consisted of a right transthoracic subtotal esophagectomy with mediastinal and cervical lymph node dissection, external esophagostomy of the neck, and gastrostomy. Forty days after the first surgery, a gastroduodenal artery- and right gastroepiploic vessel-preserving pancreatoduodenectomy with Child’s reconstruction was performed as the second-stage surgery. Esophageal reconstruction was achieved using a gastric tube via the percutaneous route with vascular anastomosis.     

Hepatectomy for liver metastasis from ampullary cancer after pancreatoduodenectomy

A 51-year-old Japanese woman with a solitary liver metastasis originating from a carcinoma of the ampulla of Vater was successfully treated by partial hepatectomy 19 months after curative pancreatoduodenectomy with lymphadenectomy. Histologic examination revealed a stage III well-differentiated tubular adenocarcinoma (pT2, pN1, and pM0). Postoperative serum concentrations of carcinoembryonic antigen increased exponentially to 133 ng/mL. The carcinoembryonic antigen doubling time was 63 days. Computed tomography and ultrasonography of the abdomen showed a solitary metastasis in segment VI of the liver. Since neither local recurrences nor other distant metastases were detected, the patient underwent partial hepatectomy. Histologic study confirmed the presence of a metastatic liver tumor from the ampullary carcinoma. The carcinoembryonic antigen levels returned to normal immediately after the partial hepatectomy. She was well without signs of recurrence 18 months after partial hepatectomy.     

The clinicopathologic and immunohistochemical characteristics of ampulla of Vater carcinoma: the intestinal type is associated with a better prognosis

BACKGROUND/AIMS: We wanted to compare the clinicopathological parameters with the immunohistochemical expression patterns and patient survival for the intestinal type (IT) and the pancreatobiliary type (PT) of ampulla of Vater carcinoma. Ampulla of Vater carcinoma can be classified histologically into either IT or PT. The biologic behavior and patient prognosis vary considerably in relation to the tumor type. METHODOLOGY: From September, 1995, to February, 2004, 34 patients with the pathologic diagnosis of ampulla of Vater carcinoma were retrospectively reviewed and the prognostic factors were analyzed. To classify the phenotypes of the tumors, the keratin types (CK7 and CK20), the type of apomucin of the mucosa (MUC2), and the glucose transporter (GLUT1) were studied for differentiating the tumor types. RESULTS: The 5-year survival rate of the 34 patients with ampulla of Vater carcinoma was 58.8%. Histologically, 12 patients had IT and 22 had PT, and the IT patients all survived. The long-term survival after resection of the tumor was significantly greater for the patients with IT than for the patients with PT. Although these differences were not statistically significant, the prognosis of IT group seemed more favorable (p = 0.0955). On the immunohistochemical staining, MUC2 (p < 0.0001), CK20 (p = 0.0002) and CK7 (p = 0.0368) were statistically effective, but not GLUT1, for differentiating IT from PT. CONCLUSIONS: For the classification of the tumor phenotypes, performing immunohistochemical staining were helpful to differentiate the two types of tumor. A study with a larger number samples would probably elucidate the different clinical course between these two types of ampulla of Vater carcinoma.     

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.     

Carcinoid of the ampulla of Vater

Endocrine neoplasms only rarely occur at the ampulla of Vater, comprising mostly carcinoids and malignant carcinoids, as well as few cases of poorly differentiated endocrine carcinomas (small cell carcinomas). Only 105 cases are reported in the literature, most as single case reports. For many years, the neoplasms of the disseminated neuroendocrine cell system of the gastrointestinal tract have been subsumed as 'carcinoids'. Instead, in the latest World Health Organization (WHO) classification published in 2000, it is recommended to distinguish between (i) well-differentiated endocrine tumors (carcinoids); (ii) well-differentiated endocrine carcinomas (malignant carcinoids); and (iii) poorly differentiated endocrine carcinomas (small cell carcinomas). Patients with carcinoid tumors of the ampulla of Vater are very often free of clinical and laboratory findings that belong to the carcinoid syndrome. Approximately 26% of all patients with carcinoid tumor reported in the literature had neurofibromatosis. Besides endoscopic retrograde cholangiopancreatography, endosonography, computed tomography or magnetic resonance imaging may complete the staging approach of this tumor. The Kausch-Whipple procedure or pylorus-preserving pancreaticoduodenectomy is considered the treatment of choice for ampullary, well-differentiated carcinoids >2.0 cm and for ampullary neuroendocrine carcinomas. However, it should be considered that long-term survival of patients with ampullary carcinoids is also reported after local tumor excision (5-year survival rate of 90%). The dilemma is that the differentiation of neuroendocrine tumors cannot be assessed intraoperatively in most cases. Therefore, considering that the 5-year survival rate in patients with neuroendocrine carcinomas of the ampulla of Vater is very low without radical resection, neuroendocrine tumors of the ampulla of Vater without definite histological differentiation should undergo extended surgery.     

KL-6 mucin expression in carcinoma of the ampulla of Vater： Association with cancer progression

AIM: To assess histochemical expression of KL-6 and its clinicopathological significance in carcinoma of the ampulla of Vater. METHODS: Ampullary carcinoma tissues were collected from 38 patients who underwent pancreatoduodenectomy or local resection. Tissues were subjected to immunohistochemical analysis using KL-6 antibody. RESULTS: Positive staining of ampullary carcinoma cells was observed in 26 (68.4%) cases. Staining was not found in the surrounding non-cancer regions of the ampullary tissues. Remarkable KL-6 expression was observed in invasive carcinoma cells in pancreatic and duodenal tissues and in metastatic carcinoma cells in lymph nodes. Positive KL-6 expression was related to lymph node metastasis (P=0.020), pancreatic invasion (P=0.016), duodenal invasion (P= 0.034), and advanced stage of TNM clinical classification (P=0.010). Survival analysis showed that positive expression of KL-6 was related to a poorer prognosis (P=0.029). CONCLUSION: The aberrant expression of KL-6 mucin is significantly related to unfavorable behaviors of carcinoma of the ampulla of Vater.     

A collision tumor composed of cancers of the bile duct and ampulla of Vater--immunohistochemical analysis of a rare entity of double cancer

Collision cancer of the bile duct and the papilla of Vater is an extremely rare entity. This is the first report of a case of bile duct collision cancer. A 75-year-old man presented with jaundice. Computed tomography showed isodensity masses in the middle bile duct and the papilla of Vater. Magnetic resonance cholangiopancreatography showed a tuberous filling defect in the middle bile duct. Gastroduodenal endoscopy showed a tumor with ulceration at the papilla of Vater. The patient was diagnosed with cancers of the middle bile duct and the papilla of Vater, and a pylorus-preserving pancreatoduodenectomy was performed. On pathological examination, the tumor in the middle bile duct showed a well differentiated carcinoma that had spread to the proximal bile duct, whereas the tumor in the papilla of Vater showed a papillo-tubular carcinoma with a marked production of mucin, suggesting an intestinal type of ampullary cancer. These tumors were directly communicated by microscopic findings. Therefore, the immunohistochemical characteristics were analyzed, using several antibodies, to determine whether the origins of the 2 cancers were different or not. As a result, it was concluded that this was a case of collision cancer of the middle bile duct and the papilla 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.