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.     

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.     

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.     

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.     

Small-cell neuroendocrine carcinoma of the ampulla of Vater

We report a patient (an 80-year-old woman) with anemia and fecal occult blood, who had an emergency operation for carcinoma of the cecum (well-differentiated adenocarcinoma without local lymph node metastasis). Postoperative magnetic resonance cholangiopancreatography, cholangiography, and upper gastroduodenal endoscopy showed a tumor of the ampulla of Vater, and pylorus-preserving pancreatoduodenectomy was performed. Histology of the resected tumor was that of small-cell carcinoma, and immunohistochemistry showed positive staining for neuron-specific enolase, chromogranin A, and synaptophysin, confirming the neuroendocrine nature of the tumor. As the histology of the tumor was distinct from cecal carcinoma, and no tumors were found in other organs, the tumor was diagnosed as primary small-cell neuroendocrine carcinoma of the ampulla of Vater. The patient died due to multiple liver metastases of the carcinoma of the ampulla of Vater 7 months after the pancreatoduodenectomy. The clinical and morphological features of this disease have been reported in nine individuals previously.     

Pancreatoduodenectomy after coronary artery bypass grafting using the right gastroepiploic artery: a case report

We herein report the case of an advanced ampullary cancer developed 80 months after coronary artery bypass grafting (CABG) using the right gastroepiploic artery (RGEA) and successfully treated by pancreaticoduodenectomy (PD) with revascularization using the great saphenous vein. A 69-year-old Japanese male was admitted for examination with one-month history of nausea and appetite loss. He underwent three vessel CABG, involving bypassing between the right coronary artery and RGEA about 80 months before. The preoperative diagnosis with CT scan and gastric endoscope was carcinoma of the papilla of Vater. Preoperative abdominal angiography showed the RGEA graft remained well patent. He underwent PD with regional lymph node dissection after revascularization of the RGEA. The postoperative clinical course was uneventful. The histopathological examinations of the resected specimen revealed adenocarcinoma of the ampulla, pT2, pN0, M0 stage IB. The patient is currently alive without any further signs of ischemic heart disease several months after his operation. This case report demonstrates that the radical PD with revascularization using other vein graft can be safely performed after CABG using the RGEA.     

Pancreatoduodenectomy for local intrapancreatic recurrence after bile duct resection of mid bile duct carcinoma

Cancers originating from the proximal and mid portions of the common bile duct exhibit a variable degree of longitudinal tumor spread, thus curative resection with attainment of sufficient resection margins is often difficult to achieve. Not surprisingly, local tumor recurrence after noncurative resection occurs frequently, but most recurrences are not considered viable indications for a second operative intervention. We describe our experience of attempting to achieve curative repeat resection after local recurrence of bile duct carcinoma. Two male patients, aged 49 and 58 years, had localized tumor recurrence in the intrapancreatic bile duct 25 and 43 months after segmental bile duct resection for proximal-to-mid duct carcinoma. Following close observation for 3-4 months to determine operability, these 2 patients underwent pylorus-preserving pancreatoduodenectomy, in which both pre-existing hepaticojejunostomy and Roux jejunal limb were effectively used. There was no further recurrence for 65 and 37 months after repeat resection, respectively. Repeat resection with pancreatoduodenectomy may be a feasible treatment modality for selected patients showing late intrapancreatic recurrence after prior segmental bile duct resection.     

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.     

Prognostic factors in patients with carcinoma of the papilla of Vater

BACKGROUND/AIMS: The prognosis after curative resection for patients with carcinoma of the papilla of Vater is relatively better than that for other peripancreatic cancer. However, prognostic factors after resection of the carcinoma have not been identified. METHODOLOGY: From 1983 to 1999, 16 patients with carcinoma of the papilla of Vater underwent standard pancreatoduodenectomy and dissection of regional lymph nodes. We followed the patients for 63 days to 17 years (median, 27 months) and analyzed clinicopathologic variables in relation to prognosis. RESULTS: The survival rate at 5 years was 50.5%. The morphologic factors predicting poor outcome were macroscopic ulcer formation and microscopic pancreatic, venous, or perineural invasion. Tumors with ulcer formation tended to infiltrate into the duodenum and pancreas, but not into veins or the perineural space. Eight of 16 patients died due to recurrence of the cancer; liver metastasis (n = 6) or peritoneal dissemination (n = 2). CONCLUSIONS: Patients with carcinoma of the papilla of Vater demonstrating ulcer formation or invasion into the pancreas, vein, or perineural space may benefit from adjuvant therapy to reduce the risk of liver metastasis. Careful observation is essential for liver metastasis or peritoneal dissemination after surgery; especially in patients with ulcer formation or venous invasion.     

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.     

The leser-trelat sign in association with carcinoma of the ampulla of Vater

A 45-yr-old woman was admitted to our hospital for surgical treatment of obstructive jaundice. She was treated by a standard pancreatoduodenectomy (Whipple procedure). The biopsy of the specimen disclosed an adenocarcinoma of the ampulla of Vater. Six months before the appearance of the obstructive jaundice, she noted a sudden onset of many seborrheic keratoses on the upper part of her trunk and upper extremities. This, to our knowledge, is the first case of Leser-Trelat sign associated with adenocarcinoma of the ampulla of Vater.     

Oncological benefit of preoperative endoscopic biliary drainage in patients with hilar cholangiocarcinoma

Due to advances in endoscopic equipment and techniques, preoperative endoscopic biliary drainage (EBD) has been developed to serve as an alternative to percutaneous transhepatic biliary drainage (PTBD). This study sought to clarify the benefit of EBD in comparison to PTBD in patients who underwent radical resections of hilar cholangiocarcinoma. One hundred and forty‐one patients underwent radical surgery for hilar cholangiocarcinoma between 2000 and 2008 were retrospectively divided into two groups based on the type of preoperative biliary drainage, PTBD (n = 67) or EBD (n = 74). We investigated if the different biliary drainage methods affected postoperative survival and mode of recurrence after median observation period of 82 months. The survival rate for patients who underwent EBD was significantly higher than those who had PTBD (P = 0.004). Multivariate analysis revealed that PTBD was one of the independent factors predictive of poor survival (hazard ratio: 2.075, P = 0.003). Patients with PTBD more frequently developed peritoneal seeding in comparison to those who underwent EBD (P = 0.0003). PTBD was the only independent factor predictive of peritoneal seeding. In conclusion, EBD might confer an improved prognosis over PTBD due to prevention of peritoneal seeding, and is recommended as the initial procedure for preoperative biliary drainage in patients with hilar cholangiocarcinoma.     

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.     

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.     

Hepatic parenchymal atrophy induction for intractable segmental bile duct injury after liver resection

Liver resection can result in various types of bile duct injuries but their treatment is usually difficult and often leads to intractable clinical course. We present an unusual case of hepatic segment III duct (B3) injury, which occurred after left medial sectionectomy for large hepatocellular carcinoma and was incidentally detected 1 week later due to bile leak. Since the pattern of this B3 injury was not adequate for operative biliary reconstruction, atrophy induction of the involved hepatic parenchyma was attempted. This treatment consisted of embolization of the segment III portal branch to inhibit bile production, induction of heavy adhesion at the bile leak site and clamping of the percutaneous transhepatic biliary drainage (PTBD) tube to accelerate segment III atrophy. This entire procedure, from liver resection to PTBD tube removal took 4 months. This patient has shown no other complication or tumor recurrence for 4 years to date. These findings suggest that percutaneous segmental portal vein embolization, followed by intentional clamping of external biliary drainage, can effectively control intractable bile leak from segmental bile duct injury.     

Case of early ampullary cancer treated by endoscopic papillectomy

We herein report a case of ampullary cancer in a 65‐year‐old man who underwent endoscopic papillectomy. Duodenoscopy revealed an exposed‐type tumor mass at the ampulla of Vater. Histology of the biopsy specimen demonstrated well‐differentiated adenocarcinoma. Endoscopic ultrasonography and intraductal ultrasonography showed a hypoechoic mass limited to the ampulla of Vater (clinical stage, T1). Endoscopic papillectomy was performed after informed consent was obtained. Histological examination of the resected specimen revealed adenocarcinoma limited to the ampulla of Vater (final stage, pT1). Both accurate preoperative T staging and proper histological evaluation of the resected specimen appear to justify endoscopic treatment of early ampullary cancer.     

Metachronous bile duct cancer nine years after resection of gallbladder cancer

We report a rare case of a 74-year-old man with metachronous gallbladder cancer and bile duct cancer who underwent curative resection twice, with the operations nine years apart. At the age of 65 years, the patient underwent a cholecystectomy and resection of the liver bed for gallbladder cancer. This was a welldifferentiated adenocarcinoma, with negative resection margins （T2NOM0, stage Ⅰ B）. Nine years later, during a follow-up examination, abdominal computed tomography and MRCP showed an enhanced 1.7 cm mass in the hilum that extended to the second branch of the right intrahepatic bile duct. We diagnosed this lesion as a perihilar bile duct cancer, Bismuth type Ⅲ a, and performed bile duct excision, right hepatic Iobectomy and Roux-en-Y hepaticojejunostomy. The histological diagnosis was a well-differentiated adenocarcinoma with one regional lymph node metastasis （TINIM0, stage Ⅱ B）. Twelve months after the second operation, the patient is well, with no signs of recurrence. This case is compared with 11 other cases of metachronous biliary tract cancer published in the world medical literature.     

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.     

Obstructive Jaundice Due to Duodenal Ulcer Induced by Lenvatinib Therapy for Hepatocellular Carcinoma

An 82-year-old man with hepatocellular carcinoma presented with upper abdominal pain, vomiting, and jaundice. He had been taking a standard lenvatinib dose for three months. Although acute cholangitis was suggested, imaging studies failed to detect the biliary obstruction site. An endoscopic examination following discontinuation of lenvatinib and aspirin revealed multiple duodenal ulcers, one of which was formed on the ampulla of Vater and causing cholestasis. Endoscopic biliary drainage and antibiotics improved concomitant Enterobacter cloacae bacteremia. Ulcer healing was confirmed after rabeprazole was replaced with vonoprazan and misoprostol. Our case shows that lenvatinib can induce duodenal ulcers resulting in obstructive jaundice.     

Biliopancreatic and biliary leak after pancreatoduodenectomy treated by percutaneous transhepatic biliary drainage

BackgroundComplementary to percutaneous intra-abdominal drainage, percutaneous transhepatic biliary drainage (PTBD) might ameliorate healing of pancreatic fistula and biliary leakage after pancreatoduodenectomy by diversion of bile from the site of leakage. This study evaluated technical and clinical outcomes of PTBD for this indication.MethodsAll patients undergoing PTBD for leakage after pancreatoduodenectomy were retrospectively evaluated in two tertiary pancreatic centers (2014–2019). Technical success was defined as external biliary drainage. Clinical success was defined as discharge with a resolved leak, without additional surgical interventions for anastomotic leakage other than percutaneous intra-abdominal drainage.ResultsFollowing 822 pancreatoduodenectomies, 65 patients (8%) underwent PTBD. Indications were leakage of the pancreaticojejunostomy (n = 25; 38%), hepaticojejunostomy (n = 15; 23%) and of both (n = 25; 38%). PTBD was technically successful in 64 patients (98%) with drain revision in 40 patients (63%). Clinical success occurred in 60 patients (94%). Leakage resolved after median 33 days (IQR 21–60). PTBD related complications occurred in 23 patients (35%), including cholangitis (n = 14; 21%), hemobilia (n = 7; 11%) and PTBD related bleeding requiring re-intervention (n = 4; 6%). In hospital mortality was 3% (n = 2).ConclusionAlthough drain revisions and complications are common, PTBD is highly feasible and appears to be effective in the treatment of biliopancreatic leakage after pancreatoduodenectomy.