Pancreatic endocrine tumors with intraductal growth into the main pancreatic duct and tumor thrombus within the portal vein: a case report and review of the literature

Pancreatic endocrine tumors are rare tumors classified into "functioning" and "nonfunctioning" tumors. A 68-year-old man was admitted to our hospital with the chief compliant of abdominal pain. Various imaging studies demonstrated a mass in the head of the pancreas with intraductal growth into the main pancreatic duct and an intraportal mass. The patient underwent a curative surgical operation. Histopathological examination revealed that it was nonfunctioning endocrine carcinoma of the pancreas. This is the first reported case of a pancreatic endocrine tumor with intraductal growth into the main pancreatic duct and tumor thrombus within the portal vein.     

Invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma without mucin hypersecretion but with changes in the pancreatic duct on pancreatography

We present a case of invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma without mucin hypersecretion in a 65‐year‐old man with a 45‐year history of alcohol abuse and a 2‐year follow‐up of chronic pancreatitis. Two years previously, in May 1998, he was admitted for investigation of abdominal pain. Computed tomography (CT) showed diffuse dilation of the main pancreatic duct with atrophy of the pancreatic parenchyma. Endoscopic retrograde pancreatography (ERP) showed a diffusely dilated main pancreatic duct with irregular side branches in the head of the pancreas. Chronic alcoholic pancreatitis was diagnosed on the basis of the pancreatography findings. The patient was readmitted for investigation of progressive weight loss in August 2000. Serum CA19‐9 levels were markedly elevated (750U/ml) and CT showed enlargement of the head and body of the pancreas. ERP showed irregularity of the main pancreatic duct in the head of the pancreas, and the distal main pancreatic duct (which was dilated on initial ERP examination) was interrupted in the body of the pancreas. Suspected pancreatic carcinoma was diagnosed, and pylorus‐preserving pancreatoduodenectomy was performed. Frozen section examination of the cut end of the pancreas revealed ductal carcinoma, and total pancreatoduodenectomy with portal vein resection was performed. Histologically, the resected tumor was diagnosed as an invasive carcinoma derived from intraductal papillary adenocarcinoma without mucin hypersecretion. We recommend observing changes in the pancreatic duct on pancreatography to diagnose invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma in a resectable state.     

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.     

Hemosuccus pancreaticus due to intraductal papillary-mucinous carcinoma of the pancreas

A 72-year-old female was referred to our hospital for evaluation of a hyperechoic mass in the pancreatic head with ultrasound sonography. She had no symptom expect slight anemia (Hb 11.3 g/dl). On endoscopy, blood was expelled from the orifice of the major duodenal papilla, but excretion of mucus was not detected. Endoscopic retrograde pancreatography revealed an irregular defect in the main pancreatic duct at the head of the pancreas. Computed tomography revealed a 2-cm mass with a low density lesion in the pancreas head. On suspicion of malignant tumor of the pancreas, pylorus-preserving pancreaticoduodenectomy was performed. Histological diagnosis was intraductal papillary-mucinous carcinoma without mucin hypersecretion. It grew within the inferior branch of the main pancreatic duct, and the top of the tumor stood out into the main pancreatic duct. As the causes of hemosuccus pancreaticus, pancreatic benign diseases, for example, chronic pancreatitis, pseudocyst, arterial aneurysm and pseudoaneurysm, are known, but pancreatic tumors are rare. In particular, this may be the first report of hemosuccus pancreaticus induced by intraductal papillary-mucinous carcinoma of the pancreas without mucin hypersecretion.     

Case of intraductal papillary mucinous tumor (noninvasive adenocarcinoma) of the pancreas resected 27 years after onset

Summary A case of intraductal papillary mucinous tumor (IPMT) of the pancreas resected 27 yr after onset is presented. In March of 1997, a 71-yr-old man was admitted to our hospital with a complaint of severe epigastric pain. He had initially undergone endoscopic retrograde pancreatography (ERP) in April 1971 in our hospital and the patient had been followed up for pancreatografic changes for 26 yr. Dilatation of the main pancreatic duct gradually progressed during follow-up, and the filing defect owing to the tumor became demonstrable. On admission, ERP revealed diffuse dilatation of the main pancreatic duct, which was 20 mm in diameter, and the filling defect of 35 mm in diameter. We diagnosed this patient as having an IPMT of the pancreas. Considering his general condition, pancreatic segmentectomy was carried out, and the postoperative course was favorable. Histological findings were compatible with those of noninvasive papillary adenocarcinoma. This is a precious case for studying the natural history of intraductal papillary tumor of the pancreas and to evaluate the application of surgery, because the biologic behavior of this tumor is much less aggressive than that of pancreatic ductal cell carcinoma.     

A case of spindle cell type anaplastic carcinoma of the pancreas -review of Japanese case report-

We present a case of spindle cell type anaplastic carcinoma of the pancreas in a 63-year-old woman. A pancreatic mass was incidentally detected by routine abdominal ultrasonography examination for her hepatitis B infection, and she was admitted to our hospital for further examination. Computed tomography revealed a hypo-vascular mass measuring 25mm in maximal dimension at the pancreas body. Endoscopic ultrasonography showed a hypoechoic mass as the pancreas body and a swollen lymph node near the tumor. Endoscopic retrograde pancreatography revealed disruption of the main pancreatic duct, and carcinoma cells were detected in pancreatic juice obtained via an endoscopic nasopancreatic drainage tube. We diagnosed this case as an invasive ductal adenocarcinoma of the pancreas body, therefore the distal pancreatectomy with splenectomy (D1+α) was performed. The histopathological diagnosis for this case was a "spindle cell type anaplastic carcinoma of the pancreas". The patient has remained well with no evidence of recurrence for 9 months since her operation.     

Intraductal carcinoma in a surgically resected pancreas with chronic pancreatitis

A 59-yr-old Japanese male presented with epigastralgia. Endoscopic retrograde cholangiopancreatography (ERCP) revealed narrowing of the inferior common bile duct and protein plugs in the main pancreatic duct. He was diagnosed as suffering from chronic pancreatitis with suspicion of a pancreatic head tumor, and a pancreatoduodenectomy was performed. Histologically, a diffuse chronic pancreatitis was evident in the resected pancreas. Although no tumors were seen in the head portion of the pancreas around the inferior common bile duct, an intraductal carcinoma was found in the second branch of Santorini’s duct. Precancerous alteration of the duct epithelium, presenting papillary hyperplasia, and atypical hyperplasia were observed in areas continuous with the intraductal carcinoma. Immunohistochemically, carcinoembronic antigen (CEA) was specifically expressed in atypical hyperplasia and intraductal carcinoma, but not in papillary hyperplasia.     

A case of intraductal papillary-mucinous carcinoma of the pancreas with the onset of acute pancreatitis

Intraductal papillary-mucinous carcinoma of the pancreas has been reported with increasing frequency. We report a case with intraductal papillary-mucinous carcinoma of the pancreas and discuss surgical treatment and current imaging modalities. A case with intraductal papillary-mucinous carcinoma was analyzed by radiological findings and clinical course. A 47-year-old man developed abdominal pain and nausea. Computed tomography showed a diffusely dilated main pancreatic duct. Duodenoscopy showed a patulous orifice of the pancreas with massive mucus secretion, but the pancreatic juice was not positive for malignant cells. Endoscopic retrograde cholangiopancreatography revealed a markedly dilated pancreatic duct extending from the body to the tail of the pancreas. Distal pancreatectomy was performed with splenectomy and lymph nodes dissection. Histopathological diagnosis was intraductal papillary-mucinous carcinoma. Endoscopic retrograde cholangiopancreatography is useful for diagnosing intraductal papillary mucin-producing tumors. To avoid unnecessary total pancreatectomy and preserve pancreatic function, intraoperative frozen section examination is widely available for the surgical treatment of intraductal papillary mucin-producing tumors.     

Long-term follow-up of intraductal papillary adenoma of the pancreas

Intraductal papillary mucinous tumors of the main pancreatic duct are often considered to be premalignant or malignant, and therefore surgical resection is recommended. We report two autopsy cases of intraductal papillary mucinous tumor of the pancreas following long-term observation. The first patient was an 84-year-old man with early gastric cancer treated by endoscopic mucosectomy. The second patient was a 77-year-old man with hepatocellular carcinoma treated by percutaneous pure ethanol injection and transarterial embolization. In both patients, endoscopic retrograde pancreatography showed a diffusely dilated main pancreatic duct, with intraductal filling defects expressing mucus, as well as dilated side branches. Obvious intramural nodules were not detected. Due to their advanced age and personal requests, both patients were managed conservatively and followed nonoperatively. In the first patient, serial pancreatograms showed progression of the pancreatic duct dilatation. Both patients died of gastric cancer, the first patient 7¹/₂ years and the second, 10 years after first presentation, respectively. Autopsies revealed extensive intraductal papillary adenoma throughout the dilated mucus-filled main pancreatic duct. However, there was no evidence of progression to adenocarcinoma. Based on these observations, we suggest that, in patients with intraductal papillary mucinous tumor of the pancreas without obvious intramural nodules, even if the tumor is in the main pancreatic duct, pancreatectomy may not be mandatory, particularly in the elderly. Received: March 21, 2001 / Accepted: July 6, 2001 Reprint requests to: T. Kamisawa     

A CASE OF TUMOR‐FORMING PANCREATITIS WITH A RAPID AND AGGRESSIVE CHANGE

An asymptomatic 51‐year‐old man was found to have a low echoic mass by ultrasonography, 12 mm in diameter, at the head of the pancreas during mass screening. Endoscopic ultrasonography revealed that the mass was hypoechoic with clear margins and without main pancreatic duct dilatation. Therefore, islet cell tumor was suspected, but pancreatic carcinoma could not be excluded. After 3 months, follow‐up ultrasonography revealed that the margin had become more clearly defined and the mass had tended to enlarge. Endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography revealed dilatation of the main pancreatic duct. A pylorus‐preserving pancreaticoduodenectomy was performed for the presumed diagnosis of pancreatic carcinoma. Histological examination revealed prominent fibrosis and destruction of the exocrine parenchyma without evidence of malignancy. The diagnosis of pancreatic carcinoma based on tendency of enlargement and dilatation of the main pancreatic duct was difficult in this case.     

Focal Ductal Branch Dilatation on Magnetic Resonance Cholangiopancreatography: A Hint for Early Diagnosis of Pancreatic Carcinoma

A 63-year-old man with a combination of early pancreatic carcinoma and an intraductal papillary adenoma was reported. A pancreatic cyst was detected by chance at the head of the pancreas by computed tomography for a follow-up study of early rectal carcinoma previously operated. Detailed studies by endoscopic retrograde pancreatography (ERP) showed irregular narrowing of the main pancreatic duct at the pancreatic body and magnetic resonance cholangiopancreatography (MRCP) revealed dilatation of ductal branches draining there. Brushing cytology of the pancreatic duct demonstrated cancer cells and total pancreatectomy was performed. Stepwise histo-pathological examinations of the specimen showed two foci of invasive carcinoma in the neck and body and multiple foci of severe dysplasia, some of which contained carcinoma in situ, in the body of the pancreas. The cystic tumor in the head of the pancreas was an intraductal papillary adenoma. In this case, the scrutiny of a pancreatic cyst including MRCP and ERP led to an early diagnosis of pancreatic cancer. Dilatation of ductal branches depicted by MRCP might be a new hint for early diagnosis of pancreatic carcinoma.     

Focal ductal branch dilatation on magnetic resonance cholangiopancreatography: a hint for early diagnosis of pancreatic carcinoma

A 63-year-old man with a combination of early pancreatic carcinoma and an intraductal papillary adenoma was reported. A pancreatic cyst was detected by chance at the head of the pancreas by computed tomography for a follow-up study of early rectal carcinoma previously operated. Detailed studies by endoscopic retrograde pancreatography (ERP) showed irregular narrowing of the main pancreatic duct at the pancreatic body and magnetic resonance cholangiopancreatography (MRCP) revealed dilatation of ductal branches draining there. Brushing cytology of the pancreatic duct demonstrated cancer cells and total pancreatectomy was performed. Stepwise histo-pathological examinations of the specimen showed two foci of invasive carcinoma in the neck and body and multiple foci of severe dysplasia, some of which contained carcinoma in situ, in the body of the pancreas. The cystic tumor in the head of the pancreas was an intraductal papillary adenoma. In this case, the scrutiny of a pancreatic cyst including MRCP and ERP led to an early diagnosis of pancreatic cancer. Dilatation of ductal branches depicted by MRCP might be a new hint for early diagnosis of pancreatic carcinoma.     

Case of intraductal papillary mucinous tumor (noninvasive adenocarcinoma) of the pancreas resected 27 years after onset.

A case of intraductal papillary mucinous tumor (IPMT) of the pancreas resected 27 yr after onset is presented. In March of 1997, a 71-yr-old man was admitted to our hospital with a complaint of severe epigastric pain. He had initially undergone endoscopic retrograde pancreatography (ERP) in April 1971 in our hospital and the patient had been followed up for pancreatografic changes for 26 yr. Dilatation of the main pancreatic duct gradually progressed during follow-up, and the filling defect owing to the tumor became demonstrable. On admission, ERP revealed diffuse dilatation of the main pancreatic duct, which was 20 mm in diameter, and the filling defect of 35 mm in diameter. We diagnosed this patient as having an IPMT of the pancreas. Considering his general condition, pancreatic segmentectomy was carried out, and the postoperative course was favorable. Histological findings were compatible with those of noninvasive papillary adenocarcinoma. This is a precious case for studying the natural history of intraductal papillary tumor of the pancreas and to evaluate the application of surgery, because the biologic behavior of this tumor is much less aggressive than that of pancreatic ductal cell carcinoma.     

Atypical ductal hyperplasia of the pancreas associated with a stricture of the main pancreatic duct

Atypical ductal hyperplasia of the pancreas is thought to be a precancerous lesion. We report a case of atypical ductal hyperplasia associated with a stricture of the main pancreatic duct. A 70-year-old man was admitted to our hospital because of abdominal pain with an elevated serum pancreatic isoamylase level. Endoscopic retrograde cholangiopancreatography disclosed a stricture of the main pancreatic duct in the body of the pancreas. Cytological evaluation of endoscopic brushings suggested adenocarcinoma. Distal pancreatec-tomy was performed. Microscopic examination of the stenotic pancreatic duct showed a hyperplastic epithelium without atypia. Atypical hyperplasia, however, was found in the distal portion of the main pancreatic duct in close proximity to the stricture. Atypical hyperplasia extended along the main pancreatic duct into the ductal branches of the pancreatic tail. In contrast to the vast majority of patients with atypical hyperplasia, the atypical hyperplasia seen in the present patient had no histological features suggestive of intraductal extension of the invasive carcinoma or intraductal papillary-mucinous tumor, thus representing a sporadic precancerous lesion, and it may have been equivalent to carcinoma in situ. Pancreatic duct stricture and the resultant stasis of the pancreatic juice may have promoted the atypical changes in the ductal cells upstream of the stricture. Received: August 20, 2001 / Accepted: February 8, 2002 Reprint requests to: M. Kogire Editorial on page 311     

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.     

Acinar cell carcinoma of the pancreas with predominant intraductal growth: report of a case

Acinar cell carcinoma (ACC) of the pancreas accounts for approximately 1% of all exocrine pancreatic tumours. We report a rare form of ACC in a 66-year-old man. This tumour was revealed by epigastric pain and weight loss. Abdominal computed tomography showed a hypodense, well-demarcated, heterogeneous lesion, in the head of the pancreas, measuring 4.2 cm in diameter. There was a marked dilatation of the main pancreatic duct upstream, with tumour spreading within this duct. The diagnosis of ACC was made on the fine needle aspiration cytology performed during endoscopic ultrasound examination. On the pancreaticoduodenectomy specimen, the dilated main pancreatic duct (2.5 cm in diameter) was filled by an exophytic tumour. Histological examination showed an ACC, with predominant intraductal growth (main and accessory pancreatic ducts), with pancreatic parenchymal and duodenal invasion. Neuroendocrine markers were negative. To our knowledge, this is the second report of an ACC with predominant intraductal spread. These rare forms of ACC can be confused with intraductal papillary-mucinous neoplasms. In our report, fine needle aspiration cytology performed during endoscopic ultrasound examination was a valuable tool in the diagnostic assessment.     

A case report of cribriform carcinoma of the pancreas with long time survival

A 62-year-old man had complained of left abdominal pain and tenderness and a body weight loss. Abdominal ultrasonography and computed tomography revealed homogeneous tumor with clear margin, and an irregular shape (3.5 x 2.0 cm) in the body of the pancreas. Endoscopic retrograde cholangiopancreatography showed a shadow defect in the main pancreatic duct adjacent to the tumor, which suggested intraductal tumor spread. Distal pancreatectomy with splenectomy and left paraaortic lymph node dissection was performed. Microscopically, the tumor showed microtubular carcinoma, which was characterized by a cribriform pattern, medullary growth, and little interstitium. The tumor was encapsulated by a relatively thick fibrous capsule. The patient was discharged uneventfully, and he is alive 33 months after operation without a distinct sign of recurrence. In conclusion, cribriform carcinoma of the pancreas has specific characteristics, such as good prognosis, expansive growth with little invasion, intraductal growth and spread without mucin production and histological marked cribriform pattern. This type of carcinoma should be classified as a new disease entity of carcinoma of the pancreas.     

Pancreatic adenosquamous carcinoma, 9 mm in size,diagnosed preoperatively by transpapillary biopsy. Report of a case

Adenosquamous carcinoma of the pancreas is a rare variant of pancreatic cancer, which is known to be difficult to diagnose. We herein report a case diagnosed by transpapillary biopsy of the pancreatic duct. A 73‐year‐old man was referred to our department with suspicion of pancreatic cancer. Ultrasound showed a hypoechoic mass, 9 mm in diameter, in the body of the pancreas. Endoscopic retrograde cholangiopancreatography demonstrated a stenosis of the main pancreatic duct in the body with upstream dilatation and a shift of the bile duct to the left. Histological examination of the specimens obtained by transpapillary biopsy of the pancreatic duct revealed both adenocarcinoma and squamous cell carcinoma. Pancreatoduodenectomy was performed and, histopathologically, the tumor was diagnosed as adenosquamous carcinoma of the pancreatic body, 9 mm in size. This is the first case of adenosquamous carcinoma of the pancreas preoperatively diagnosed by transpapillary biopsy of the pancreatic duct, and the smallest stage I (TNM classification) case of such carcinoma detected to date.     

Intraductal polypoid growth variant of pancreatic acinar cell carcinoma metastasizing to the intrahepatic bile duct 6 years after surgery: A case report and literature review

We present the first reported case of intraductal polypoid growth (IPG) variant of pancreatic acinar cell carcinoma (ACC) metastasizing to the intrahepatic bile duct. A 58-year-old Japanese woman had previously presented with obstructive jaundice and a 7.0 cm mass in the pancreatic head. She underwent biliary drainage for 2 months followed by pancreatectomy. Histological examination revealed a carcinoma with acinar pattern, immunohistochemically positive for trypsin, and acinar cell carcinoma was diagnosed. IPGs were prominent in the main pancreatic duct and its tributaries, extending into the intrapancreatic bile duct with tumor casts in the lumen. Imaging examinations 6 years later revealed a growing lesion within the intrahepatic bile duct. Needle biopsy examination suggested metastasis of ACC, and she underwent chemoradiation therapy and partial hepatectomy. Histological examination demonstrated ACC confined to the intrahepatic bile duct. The localization of metastasis and slow growth may indicate indolent biologic behavior of the IPG variant.     

Pancreatic tumor associated with pancreas divisum

BACKGROUND: Although there has been considerable controversy regarding pancreas divisum and pancreatitis, little discussion of this has taken place. The purpose of the present paper was to investigate the relationship between these two conditions. METHODS: A retrospective investigation was undertaken of pancreatic tumors associated with pancreas divisum, in 650 cases of pancreatic carcinoma, 80 cases of intraductal papillary mucinous tumor of the pancreas and 32 cases of pancreas divisum. RESULTS: Of these 32 cases, four (12.5%) were associated with pancreatic tumor: pancreatic carcinoma (n = 3) and intraductal papillary mucinous tumor (n = 1). All tumors developed from the dorsal pancreas of pancreas divisum. Periductal and interlobular fibrosis detected in the non-carcinomatous pancreas of the margin of distal pancreatectomy implied that chronic dorsal pancreatitis associated with pancreas divisum preceded carcinoma. CONCLUSIONS: Pancreatic tumors were detected in 12.5% of cases of pancreas divisum. In pancreas divisum, longstanding pancreatic duct obstruction caused by relative stenosis of the minor duodenal papilla might be a factor promoting oncogenesis.