A case of adenosquamous carcinoma of the papilla of Vater]

Adenosquamous carcinoma of the papilla of Vater is a rare tumor and only a few cases have been reported so far. Here, we report a case of adenosquamous carcinoma in a 76-year-old male who presented with jaundice and right upper quadrant abdominal pain. Ultrasonography and enhanced abdominal CT scans showed dilated common bile duct (CBD) and intrahepatic bile duct (IHD) with a suspicious obstructing mass in distal CBD. On endoscopy, obstructing and ulcerated mass was noted on the papilla of Vater. Histopathological inspection of the biopsied specimens from mass showed adenosquamous cell carcinoma of the papilla of Vater. Since the patient refused operation, we inserted a self-expandable metallic stent in distal CBD. This is the first case report on adenosquamous carcinoma of the papilla of Vater in Korea.     

Resectable gallbladder cancer presenting with acute pancreatitis caused by hemobilia

A 79-year-old female was transferred to our hospital because of suspicion that her acute pancreatitis was caused by stone impaction in the common bile duct (CBD). Laboratory examination showed aspartate aminotransferase, 1645 U/l; alanine aminotransferase, 476 U/l; amylase, 1365 U/l; and white blood cells, 10700/μl. Computed tomography (CT) showed an enhanced tumor in the neck of the gallbladder, an abnormal CBD filled with a high-density area, and localized swelling in the head of the pancreas. Magnetic resonance cholangiopancreatography also showed a low-intensity area in the CBD. Endoscopic retrograde cholangiopancreatography showed coagulated blood discharged from the papilla of Vater. The diagnosis was acute pancreatitis caused by impaction of coagulated blood from a gallbladder tumor. A curative operation was performed 10 days after endoscopic bile duct drainage. Gallbladder cancer (GBCa) has no special symptoms and is usually diagnosed at an advanced stage; however, hemobilia and acute pancreatitis are unusual as an initial presentation of GBCa.     

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.     

Villous tumor of the papilla of Vater presenting with hypopotassemia

We report a case of villous tumor of the papilla of Vater associated with hypopotassemia. The patient was a 73-year-old woman who presented with jaundice and fever. She had a history of diabetes mellitus and liver dysfunction. Laboratory studies revealed that levels of total bilirubin, alkaline phosphatase, and C-reactive protein, and the white blood cell count were elevated (suggestive of cholangitis) and that the serum potassium level was markedly reduced, to 1.9 mEq/l (normal value 3.5–5.0 mEq/l). Duodenoscopy showed a villous tumor arising in the papilla of Vater. Percutaneous transhepatic biliary drainage was performed. Approximately 700–1500 ml of bile with viscous mucoid fluid was drained daily. Percutaneous transhepatic cholangioscopy showed a papillary lesion in the distal common bile duct. Biopsied specimens from both percutaneous transhepatic cholangioscopy and duodenoscopy disclosed tubulovillous adenoma. Endoscopic ultrasonography showed that the tumor had spread to the main pancreatic duct as well as to the common bile duct. The patient underwent pylorus-preserving pancreaticoduodenectomy. Pathology examination disclosed well differentiated adenocarcinoma, carcinoma in situ, in tubulovillous adenoma. The cancer cells were observed at the bottom of the tumor spreading in the common bile duct. This is a rare case of a patient presenting with hypopotassemia associated with a tubulovillous tumor of the papilla of Vater that secreted mucoid material.     

Local resection of a gangliocytic paraganglioma near the papilla of Vater

A 69-year-old man was admitted to our hospital with epigastric discomfort. Upper gastrointestinal endoscopy showed a submucosal tumor near the papilla of Vater. Abdominal CT and MRI showed a small, well-enhanced tumor. Endoscopic tumor biopsy was performed before the operation, but pathologic findings showed normal duodenal musosa. Nevertheless, since malignancy could not be ruled out, we resected the tumor with the sphincter of the papilla of Vater, followed by plasty of the orifice for the common bile duct and main pancreatic duct. We identified 3 parts with tumor cells; epithelioid cells, spindle cells, and ganglion-like cells. The tumor was diagnosed as gangliocytic paraganglioma of the duodenum. Treatment by resecting the tumor with the sphincter of the papilla of Vater, followed by the plasty of the orifice for the common bile duct and main pancreatic duct, was selected considering the patient's safety and to achieve radical cure.     

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vater’s papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vater’s papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.     

Recurrence of mucosal carcinoma of the bile duct, with superficial flat spread, 12 years after operation

We report herein a case of recurrent mucosal cancer of the extrahepatic bile duct, with superficial flat spread, 12 years after operation. A 67‐year‐old woman had undergone common bile duct (CBD) resection and Roux‐en‐Y reconstruction. Histologically, the tumor was papillary adenocarcinoma, with superficial flat spread, with no invasive component. The epithelium at the distal margin had been exfoliated, so the absence or presence of any remnant cancerous lesion was unclear. But the superficial flat spread had expanded to within at least 3 mm from the distal margin. About 12 years postoperatively, she was hospitalized with upper abdominal pain, and duodenoscopy demonstrated a tumor in the second portion of the duodenum. Biopsy identified adenocarcinoma. Computed tomography showed a low‐density mass between the duodenum and pancreatic head. Pancreatoduodenectomy was performed. Histologically, papillary adenocarcinoma was found within the whole of the intrapancreatic bile duct, and its histological appearance resembled that of the original tumor. Moderately differentiated tubular adenocarcinoma had invaded around the tissue of the intrapancreatic CBD. These findings suggest that remnant intramucosal flat carcinoma within the intrapancreatic bile duct had developed into invasive carcinoma over the course of 12 years. This case suggests that remnant intraepithelial flat carcinoma within the CBD may develop a late local recurrence.     

Endoscopic manometry of common bile duct and the sphincter of Oddi in patients with biliary tract stones and in controls]

Endoscopic manometry using microtransducer was applied to evaluate the motor activity of the sphincter of Oddi (SO) and common bile duct (CBD) in patients with biliary tract stones and in controls. CBD pressure, SO pressure and the incidence of irregular phasic wave pattern (IRPWP) were relatively higher in patients with biliary tract stones than those in controls. There was no significant difference in CBD and SO pressure between patients with dilated CBD and patients with non-dilated CBD, however the incidence of IRPWP was significant higher in patients with dilated CBD than that in patients with non-dilated CBD. SO pressure and the incidence of IRPWP were significant higher in patients with papillitis than those in patients without it. Patients with valvular orifice of the papilla of Vater showed high incidence of IRPWP, and in such cases it was suggested that SO had different motor functions for CBD and pancreatic duct because these ducts were seen opening onto the papilla separately according to the findings of pancreatocholangiogram. We concluded that endoscopic manometry of CBD and the SO was useful to evaluate the motor activity of SO and provided a basis for the assessment of the motor function mechanism of SO. Our results indicated the motor activity of SO had close relationship with biliary tract stones, CBD dilatation and endoscopic findings of the papilla of Vater.     

Characteristic Findings of Endoscopic Retrograde Cholangiopancreatography in Autoimmune Pancreatitis

Background/Aims

Diffuse or segmental irregular narrowing of the main pancreatic duct (MPD), as observed by endoscopic retrograde cholangiopancreatography (ERCP), is a characteristic feature of autoimmune pancreatitis (AIP).

Methods

ERCP findings were retrospectively examined in 40 patients with AIP in whom irregular narrowing of the MPD was detected near the orifice. The MPD opening sign was defined as the MPD within 1.5 cm from the orifice being maintained. The distal common bile duct (CBD) sign was defined as the distal CBD within 1.5 cm from the orifice being maintained. Endoscopic findings of a swollen major papilla and histological findings of specimens obtained from the major papilla were examined in 26 and 21 patients, respectively.

Results

The MPD opening sign was detected in 26 of the 40 patients (65%). The distal CBD sign was detected in 25 of the 32 patients (78%), which showed stenosis of the lower bile duct. The patients who showed the MPD opening sign frequently showed the distal CBD sign (p=0.018). Lymphoplasmacytic infiltration, but not dense fibrosis, was histologically detected in biopsy specimens obtained from the major papilla.

Conclusions

On ERCP, the MPD and CBD adjacent to the major papilla are frequently maintained in patients with AIP involving the pancreatic head. These signs are useful for diagnosing AIP on ERCP.     

Twelve years survival with repeated hepatectomy and lung resection for metastasis from carcinoma of the papilla of Vater after pancreaticoduodenectomy

We present a 60-year-old woman who underwent three times hepatectomy and lung resection for metastasis originating from a carcinoma of the papilla of Vater after pancreaticoduodenectomy with lymphadenectomy during 12 years. Pancreaticoduodenectomy was performed in 1980 and histological examination of original tumor revealed a stage IIA papillary adenocarcinoma (pT3, pN0, pM0). Repetitive hepatectomy underwent in 1986 (S7,8), 1988 (S2), 1991 (S4) and bilateral partial resection of lung (right S1, left S2.3) in 1990. She died from multiple skin, bone and lung metastases 12 years after pancreaticoduodenectomy. The current case is very rare, however, if patients with carcinoma of the papilla of Vater have localized liver metastases and no local recurrence, liver metastases should be resected to improve the chances for long-term survival.     

Intraductal papillary adenocarcinoma with mucin hypersecretion and coexistent invasive ductal carcinoma of the pancreas with apparent topographic separation

We report a 66-year-old man who had a cystic intraductal papillary adenocarcinoma containing a papillary adenoma, in the head of the pancreas and a coexistent invasive, well differentiated solid tubular adenocarcinoma in the tail of the pancreas. He was hospitalized with acute epigastralgia. Computed tomography demonstrated a multilocular cystic mass in the head of the pancreas and a solid tumor in the tail. Endoscopic retrograde pancreatography showed mucin secretion from an enlarged papilla of Vater, marked dilatation of the main pancreatic duct in the head and body, cystic dilatation of the uncinate branch, and irregular narrowing of the main pancreatic duct in the tail. Total pancreatectomy was performed. Between the cystic tumor and the solid tumor there was a distance of 4.8 cm of normal pancreatic parenchyma and duct, recognized both grossly and microscopically. The patient died 35 months after the operation. At autopsy, peritonitis carcinomatosa was found in the abdominal cavity. Microscopically, disseminated nodules were also well differentiated tubular adenocarcinoma. The apparent anatomic separation of these two tumors within the pancreas is extremely unusual.     

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.     

Pathogenesis of carcinoma of the papilla of Vater

Most adenomas and carcinomas of the small intestine and extrahepatic bile ducts arise in the region of the papilla of Vater. In familial adenomatous polyposis (FAP) it is the main location for carcinomas after proctocolectomy. In many cases symptoms due to stenosis lead to diagnosis at an early tumor stage. In about 80%, curative intended resection is possible. Operability is the most relevant prognostic factor. Most ampullary carcinomas resp. carcinomas of the papilla of Vater develop from adenomatous or flat dysplastic precursor lesions. They can be sited in the ampulloduodenal part of the papilla of Vater, which is lined by intestinal mucosa. They also can develop in deeper parts of the ampulla, which are lined by pancreaticobiliary duct mucosa. Intestinal-type adenocarcinoma and pancreaticobiliary-type adenocarcinoma represent the main histological types of ampullary carcinoma. Furthermore, there exist unusual types and undifferentiated carcinomas. Many carcinomas of intestinal type express the immunohistochemical marker profile of intestinal mucosa (keratin 7?, keratin 20+, MUC2+). Carcinomas of pancreaticobiliary type usually show the immunohistochemical profile of pancreaticobiliary duct mucosa (keratin 7+, keratin 20?, MUC2?). Even poorly differentiated carcinomas, as well as unusual histological types, may conserve the marker profile of the mucosa they developed from. These findings underline the concept of histogenetically different carcinomas of the papilla of Vater which develop either from intestinal- or from pancreaticobiliary-type mucosa of the papilla of Vater. Molecular alterations in ampullary carcinomas are similar to those of colorectal as well as pancreatic carcinomas, although they appear at different frequencies. In future studies, molecular alterations in ampullary carcinomas should be correlated closely with the different histologic tumor types. Consequently, the histologic classification should reflect the histogenesis of ampullary tumors from the two different types of papillary mucosa.     

Exceptional ultra-late recurrence of papilla Vater carcinoma after pancreaticoduodenectomy: two cases at 17 and 25 years

Curative resection does not always equate with long-term survival. Cancer of the papilla Vater can remain clinically quiescent for decades prior to regional or distant recurrence. Nevertheless, late and ultra-late recurrence (respectively 10 and 15 years after initial treatment) are exceptional events. This protracted disease-free interval challenges the concept of a "cure" for cancer of the papilla Vater. In the first case reported here, a 74-year-old female underwent pancreaticoduodenectomy in 1985 for cancer of the papilla Vater revealed histologically as a well-differentiated papillotubular adenocarcinoma, stage IA (UICC classification). Multiple hepatic recurrences were found 17 years after the operation; hepatic biopsy showed histologically well to moderately differentiated papillotubular adenocarcinoma. She died about 17.5 years after the original operation. The second case is that of an 82-year-old female who underwent pancreaticoduodenectomy in 1974 for cancer of the papilla Vater, histologically a well-differentiated adenocarcinoma, stage IA. In the 25.5 years after the operation, the cancer recurred at the choledochoduodenal anastomosis and involved the liver hilus. Autopsy showed histologically well to moderately differentiated adenocarcinoma at the locations indicated, together with lung and lymph node metastases. The ultra-late recurrences (>15 years) in these cases are highly exceptional and, to our knowledge, this is the first report of such recurrences in cancer of the papilla Vater. It can occur in any patient, with or without identifiable risk factors. Because cancer of the papilla Vater can recur in many prognostically favorable cases after prolonged disease-free intervals, the possibility of delayed recurrence should not be ignored.     

A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction--malignant paraganglioma?

The present case report demonstrates the history of a 50-year-old man with a mixed endocrine-neurogenous tumor of the ampulla of Vater. The tumor was localized endoscopically after an attack of melena. There were no signs of endocrinopathy. A local resection with suturing of the pancreatic duct was performed. Morphologically, there were two different tissue types (neurogenous and carcinoid-like) with numerous cells and nerve fibers reacting immunohistochemically with somatostatin and neurotensin antisera: some immunoreactivity to PP-antibodies was observed. Still, after 20 months, the patient seems to have been cured by local resection.     

"Duodenal intussusception" due to adenoma of the papilla of Vater

The case of a 55-year-old woman with a pedunculate adenoma of the papilla of Vater is presented. Diagnostic imaging modalities including ultrasonography, CT scan, magnetic resonance of cholangiopancreatography, simultaneous duodenography and cholangiography, and angiography showed a giant tumor protruding intraluminally and moving forward in the duodenum by peristalsis. It had a duodenal intussusception-like appearance, with remarkable left-lower deviation of the common bile duct and major pancreatic duct in the papilla of Vater as far as the left side of the aorta. Episodes of jaundice or ileus were absent, probably because the tumor was mobile in the duodenum. As biopsy specimens showed no malignancy and intraductal ultrasonography in the common bile duct revealed no intraductal invasion of the tumor in the papilla of Vater, the patient underwent transduodenal papillectomy with papilloplasty with pancreatic ductoplasty. Pathological diagnosis of consecutive specimens was a papillary adenoma with moderate atypia and occasional tubular structure. There seems to be an exceptional subtype of the tumor in the papilla of Vater, like this case, demonstrating the duodenal intussusception-like appearance without prominent clinical symptoms.     

Endoscopic retrograde brush cytology (ERBC) of the biliary and pancreatic ducts.

A new device with a brushing tube that can be introduced through the biopsy channel of the duodenoscope (Olympus JF B) used for cannulation of the papilla of Vater is reported. In 17 patients in whom ERCP had demonstrated ductal abnormalities, an endoscopic retrograde brush cytology (ERBC) was performed. A cytological diagnosis of malignancy was made in 8 patients and a possible malignancy in 2 cases. Seven cases were operated upon, and a malignant tumor was found in 6. In one case a malignant tumor was found at autopsy. Histological examination was performed in 6 cases and showed adenocarcinoma in all.. Two patients with malignant cells and ERCP-findings of suspected pancreatic carcinoma were not operated upon. The tumours were localized in 1 case to the papilla of Vater, in 2 to the biliary duct, in 4 to the pancreatic head, and in 2 cases to the pancreatic corpus. No complications occured. It is concluded that this technique provides information supplementary to that of other diagnostic procedures.     

Common bile duct stones: management strategies in the laparoscopic era

We reviewed our experience with the treatment of common bile duct (CBD) stones in 70 patients by sequential endoscopic-laparoscopic management and single-stage laparoscopic treatment during the past 7 years. The advantages, disadvantages, and feasibility of the two procedures are discussed to elucidate therapeutic strategies for patients harboring gallbladder stones and associated choledocholithiasis. In 44 patients, sequential endoscopic-laparoscopic management was indicatedd, and was successful in 37 of them but, in seven patients endoscopic stone extraction could not be accomplished. Single-stage laparoscopic treatment was attempted in 26 patients. In practice, laparoscopic transcystic common duct exploration or choledochotomy may not always be feasible if the cystic duct or CBD are not dilated; there is a high risk of intraoperative CBD injury in such circumstances. Laparoscopic management was considered to be especially useful for the treatment of numerous, large or difficult stones, because stone removal could be succesfully performed without any injury to the papilla of Vater. This last issue is of particular importance in patients with dilated CBD, because insufficient opening of the ampulla of Vater made by endoscopic sphincterotomy (EST) may lead to stasis and reflux-related complications such as cholangitis and recurrent stones. We conclude that the most rational management of CBD stones should be decided according to the size of the CBD, which depends on the size, number, and location of stones. Patients with dilated CBD are indicated to under-go laparoscopic single-stage treatment and combined endoscopic-laparoscopic treatment may be best for patients with non-dilated CBD.     

Somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease

We report a case of somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease (VRD). A 64-year-old woman who had had recurrent attacks of acute pancreatitis and cholangitis was found, on gastroduodenal endoscopy, to have a tumor of the papilla of Vater and multiple submucosal tumors of the stomach and duodenum. Numerous submucosal tumors were observed in the stomach, duodenum, and jejunum, and total excision of the papilla of Vater and resection of the duodenal and jejunal submucosal tumors was performed. The tumor of the papilla of Vater showed the histologic appearance of a dense proliferation of tumor cells in acinar form, from the duodenal mucosa to the muscle layer, and psammoma bodies were revealed within the tumor. Immunohistologically, the tumor cells were intensely positive for somatostatin. The submucosal tumors of the duodenum and jejunum were negative for smooth muscle actin, s-100, and neuron-specific enolase (NSE), and positive for CD34 and c-kit, and they were diagnosed as gastrointestinal stromal tumors (GISTs) according to the strict definition. The only 25 cases of papilla of Vater somatostatinoma associated with VRD to have been reported in the English-language literature since 1982 are reviewed, as well as our own case. Received: January 22, 2001 / Accepted: August 10, 2001 Reprint requests to: M. Usui     

Malignant carcinoid tumor in the papilla of Vater: case report and review of 25 cases reported in Japan

Carcinoid tumors in the papilla of Vater are rare. We describe a 48-year-old male who was diagnosed with a tumor consisting of atypical cells in the papilla of Vater. He underwent curative resection of the tumor by pancreatico-duodenectomy (PD), with dissection of the regional lymph nodes. Microscopic examination revealed a metastatic lymph node in the pancreatic region. Characteristic findings on histopathological and electron microscopic studies led to the final diagnosis of malignant carcinoid tumor. It is clear from the literature that carcinoid tumors in the papilla of Vater metastasize to the regional lymph nodes and the liver. However, it has been difficult to preoperatively diagnose carcinoid tumor in the papilla of Vater. Therefore, if we observe atypical cells in the papilla of Vater, malignant carcinoid tumor should be considered. If this tumor is suspected, PD or pylorus-preserving PD, together with dissection of the regional lymph nodes, at least along the hepatoduodenal ligament, should be considered as the treatment of first choice.