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.     

A Case of Small Pancreatic Cancer Diagnosed by Serial Follow-up Studies Promptly by a Positive K-ras Point Mutation in Pure Pancreatic Juice

We report a 74-yr-old woman who was referred to our hospital because of abdominal fullness. ERP showed a questionable irregularity of the main pancreatic duct at the body. Examination of pure pancreatic juice was positive for K-ras point mutation at codon 12 and negative for cytology. Because neither US nor CT showed apparent lesions in the pancreas, we decided to follow up the patient with serial ERP and pure pancreatic juice studies at 3-month intervals. No changes had been seen up to 18 months later, when cytology was conclusive for malignancy with an apparent stenosis of the main pancreatic duct at the body. Distal pancreatectomy with splenectomy was performed. A round mass, 12 mm in diameter, was found in the body, which proved to be an adenocarcinoma at histological examination. No extrapancreatic extension and metastases were noted. Although positive K-ras point mutation has been reported in some cases of adenoma or mucinous cell hyperplasia of the pancreas and chronic pancreatitis, our case, along with previous reports, indicated the importance of testing K-ras point mutation in pure pancreatic juices for the diagnosis of pancreatic cancer at an early stage.     

Autoimmune pancreatitis starting as a localized form

Ultrasonography showed a hypoechoic mass in the head of the pancreas, and endoscopic retrograde pancreatography (ERP) showed localized stenosis of the pancreatic duct in the head of the pancreas. Computed tomography (CT) showed enlargement, with a capsule-like rim, in the head of the pancreas. Internal biliary tube drainage was performed to relieve the obstructive jaundice. The patient was followed-up under the tentative diagnosis of localized "mass-forming" pancreatitis. Four months after the drainage, CT showed diffuse swelling of the pancreas, with a capsule-like rim, and ERP demonstrated diffuse irregular narrowing of the pancreatic duct. Glucose intolerance was noted for the first time. Steroid was given as a diagnostic treatment for autoimmune pancreatitis. Two months after initiation of the steroid treatment, the ERP findings were normal, and CT showed a normal pancreas. The biliary tube was removed, and the glucose intolerance was subsequently alleviated. To summarize, we report a case of autoimmune pancreatitis starting as localized "mass-forming" pancreatitis with a peripheral rim on imagings. It is very important to be well aware of the presence of the localized form of autoimmune pancreatitis. Received: July 4, 2000 / Accepted: February 2, 2001     

Dynamic CT scanning of pancreatic duct after secretin provocation in pancreas divisum

Variations in pancreatic duct diameter at CT scanning and serum pancreatic amylase response following secretin administration were studied in 29 patients with pancreas divisum and unexplained upper abdominal pain. Eleven healthy individuals were used as controls. At endoscopie retrograde pancreatography (ERP) six patients had signs of marked and six moderate pancreatitis, whereas there were no pancreatitis changes in 17 of the patients. At CT scanning patients with marked pancreatitis (ERP) had significantly increased pancreatic duct diameter as compared to patients without signs of pancreatitis. The duct was visualized in 52% of all patients before and 71% after secretin stimulation the corresponding figures for healthy controls, being 18% both before and after secretin. In patients without signs of pancreatitis, it was demonstrated in 5/17 (29%) before and 11/17 (65%) after secretin, whereas it was seen in 10/12 (83%) pancreatitis patients both before and after the hormonal provocation. In five of the nonpancreatitis patients in whom the duct was measurable before and at all study intervals (10, 20, and 50 min) after secretin, there was a significant duct dilation response both at 10 min and when comparing the maximal duct diameter after secretin to the initial values. In contrast secretin did not affect the duct caliber in pancreatitis patients. Serum pancreatic amylase increased significantly after secretin administration to healthy controls and nonpancreatitis patients but was uninfluenced in the marked and moderate pancreatitis groups, respectively. However, when all pancreatitis patients were grouped together, the amylase levels were significantly elevated by secretin. In conclusion, secretin provocation caused duct dilation at CT scanning in pancreas divisum patients without signs of pancreatitis at ERP. In this group secretin also increased the number of patients with visualized pancreatic duct. However, in pancreas divisum patients with pancreatitis and in healthy controls no such findings were observed. Secretin stimulation increased samylase in a nonspecific way. If secretin at CT scanning causes dilation of the pancreatic duct compared to its initial measurable width or visualization of an initially not demonstrable duct in patients with unexplained upper abdominal pain, the presence of pancreas divisum without morphologically manifest pancreatitis should be considered.     

Successful long-term exocrine and endocrine function of the autotransplanted pancreas in humans

Segmental pancreatic autotransplantation has been performed to prevent the severe metabolic complications of total pancreatectomy. To date 15 segmental pancreatic autotransplants have been reported, 11 of which have been performed for relief of the abdominal pain of chronic pancreatitis. The major problem with segmental pancreatic graft relates to the handling of the pancreatic duct and its secretion. In all the reported cases, the autotransplanted duct was either ligated, stapled, or occluded with synthetic polymers. In this article we present a patient who has undergone a total pancreatectomy with segmental pancreatic autotransplantation and subsequent Roux-en-Y anastomosis to the transplanted duct. Physiologic studies indicate normal endocrine function 7 years following transplant. The patient is insulin-independent and tolerates a normal meal, requiring no oral pancreatic enzyme supplementation. To our knowledge this is the first long-term report of a patient with an autotransplanted pancreas who is presently both insulin sufficient and with intact exocrine function.     

Incidence of the focal type of autoimmune pancreatitis in chronic pancreatitis suspected to be pancreatic carcinoma: experience of a single tertiary cancer center

Objective. With an increase in autoimmune pancreatitis (AIP) being reported, the focal type of AIP, which shows localized narrowing of the main pancreatic duct and focal swelling of the pancreas, has recently been recognized. Therefore, cases of focal-type AIP subjected to surgical intervention for presumptive malignancy might previously have been diagnosed as mass-forming chronic pancreatitis. The aim of this study was to elucidate the incidence of focal-type AIP in resected chronic pancreatitis at a single tertiary cancer center. The clinical and radiological features of focal-type AIP were also evaluated. Material and methods. We re-evaluated 15 patients who underwent pancreatic resection with a presumed diagnosis of pancreatic ductal adenocarcinoma, and who in the past had been diagnosed pathologically as having chronic pancreatitis. Results. Seven of 15 patients showed AIP, and the other 8 patients were diagnosed as having mass-forming chronic pancreatitis not otherwise specified by pathological retrospective examination. In other words, nearly half of the cases of resected chronic pancreatitis that were suspected to be pancreatic carcinoma preoperatively showed focal-type AIP. Regarding the characteristic findings of focal-type AIP, narrowing of the pancreatic duct on endoscopic retrograde pancreatography (ERP) might be diagnostic. Conclusions. Focal-type AIP is not a rare clinical entity and might be buried in previously resected pancreatic specimens that in the past were diagnosed simply as mass-forming pancreatitis.     

Pancreatic ductal morphological pattern and dilatation in postoperative abdominal pain in patients with congenital choledochal cyst: an analysis of postoperative pancreatograms

BACKGROUND: Postoperative abdominal pain associated with pancreatitis has been reported as a pancreas-associated complication after total biliary excision. The aim of the present study was to determine pancreatic ductal patterns in the head of the pancreas and evaluate pancreatic ductal dilatation in patients presenting with postoperative abdominal pain after radical biliary excision for congenital choledochal cyst. METHODS: Postoperative endoscopic retrograde pancreatography (ERP) was performed in a total of 38 patients (27 female and 11 male) with a history of postoperative abdominal pain or an increase in serum and urinary amylase on laboratory analysis. Pancreatic ductal configuration and ductal dilatation were determined. RESULTS: Pancreatic configurations included the usual type (n = 20), ansa pancreatica type (n = 11), loop type (n = 5), and divisum type (n = 2). Dilatation of the pancreatic duct was observed in 34 patients. The common channel was dilated in 29 patients. Mild postoperative pancreatitis occurred in 4 patients in the long term. Protein plugs were observed in the dilated pancreatic duct. CONCLUSIONS: Pancreatic ductal morphological pattern and ductal dilatation, possibly caused by long-standing stagnation of the pancreatic juice, may be associated with postoperative abdominal pain and pancreatitis in congenital choledochal cyst.     

Clinical relevance of an unfused pancreatic duct system.

In man, the main pancreatic duct is normally derived from ventral and dorsal embryological buds of the pancreas. In a minority of people, failure of fusion of the two buds results in separate drainage of the dorsal and ventral pancreas, so that the accessory duct provides the main drainage for the gland. Patients with this anomaly demonstrated at endoscopic retrograde pancreatography (ERP) have been investigated to assess whether non-fusion of the main pancreatic duct predisposes to the development of pancreatitis. A failure of fusion of the pancreatic ducts was seen in 21 out of 449 (4.7%) successful pancreatograms; four of these 21 patients had definite clinical evidence of pancreatitis and two patients had possible pancreatic disease, but in the remainder the anomaly was not considered to be clinically relevant. An abnormal pancreatogram suggesting pancreatitis was present in 116 out of the 428 patients (27.1%) with a normally fused duct system. The anomaly was found as frequently in the whole series as it was seen in patients with pancreatitis. These findings suggest that embryological failure of pancreatic duct fusion does not predispose to the development of pancreatitis. However, the presence of this anomaly may lead to misinterpretation of ultrasonographic and CT scan findings.     

Treatment of acute pancreatitis caused by calculous obstruction of the pancreatic duct by ultrasound-guided percutaneous drainage.

In a small percentage of patients with acute pancreatitis, recurrent attacks of pain and hyperamylasaemia occur when feeding is commenced. Recurrences of this type may occur because the pancreas is still swollen and inflamed, and indicate the need for a longer period of "pancreatic rest" before food is introduced. Alternatively, they may reflect the presence of "mechanical" factors leading to the recurrent pancreatitis, such as a gallstone in the common bile duct, a pseudocyst of the pancreas, or pancreatic duct obstruction. Successful resolution of the pancreatitis may require treatment of underlying causative factors. A stone in the pancreatic duct (probably a gallstone) was found to be the cause of recurrent acute pancreatitis in an elderly patient with severe cardiovascular disease, who was unfit for surgery. Pancreatitis settled after percutaneous drainage of the pancreatic duct, the technique described.     

Gastric duplication communicating with a pancreatic duct. Report of a case

A case of gastric duplication communicating with the pancreatic duct system is reported. The clinical presentation consisted of abdominal pain with recurrent acute pancreatitis. The histopathological examination showed that the duplication was lined with normal pyloric mucosa. In the tubular structure, connecting the duplication to the pancreatic ducts, an increase in the number of serotonin-positive cells was found. The pancreas showed dilatation of the duct system and chronic pancreatitis.     

Autoimmune Pancreatitis as a New Clinical Entity (Three Cases of Autoimmune Pancreatitis with Effective Steroid Therapy)

The most common forms of chronic pancreatitisare related to alcohol ingestion, whereas the entity ofnon-alcohol-associated (idiopathic) pancreatitis ispoorly understood. Autoimmunity has been suggested as a possible etiologic factor of idiopathicchronic pancreatitis. A total of 362 Japanese patientsunderwent endoscopic retrograde pancreatography (ERP)for suspected pancreatic disease, and 161 were diagnosed with chronic pancreatitis. Among them, we foundthree cases (1.86% incidence) of unique chronicpancreatitis, in which ERP revealed diffuse narrowing ofthe main pancreatic duct with an irregular wall. We diagnosed these three patients as havingpancreatitis associated with an autoimmune mechanismmorphologically and biochemically and started them onsteroid therapy. The characteristics of the these three patients were as follows:hypergammaglobulinemia, eosinophilia, ultrasonographyshowing hypoehoic diffuse swelling in the pancreas(sausage-like appearance), ERP showing diffuse narrowingof the main pancreatic duct with irregular like thumbprintlike marks,reversible exocrine insufficiency, and positiveanti-carbonic anhydrase II antibody. After one month ofthe treatment with steroids, pancreatitis dramatically improved morphologically and enzymatically.Here we describe these cases of the suspected autoimmunechronic pancreatitis. We must recognize the concept andthe features of autoimmune pancreatitis in order to avoid unnecessary surgery as pancreaticcancer.     

Santorinicele as a Cause of Chronic Pancreatic Pain

The role pancreas divisum plays in recurrent pancreatitis and chronic pancreatic pain remains controversial. When pancreatic disease does occur secondary to pancreas divisum, the pathogenesis is thought to the stenosis of the accessory duct with a resulting increase inductal pressure.
A case is reported in which stenosis of the accessory papilla orifice is thought to be responsible for cystic dilatation of the terminal portion of the duct of Santorini in a patient with pancreas divisum. This resulted in chronic pancreatic pain that resolved after sphincterotomy of the accessory papilla.     

Comparison of Regional Pancreatic Tissue Fluid Pressure and Endoscopic Retrograde Pancreatographic Morphology in Chronic Pancreatitis

The relation between pancreatic tissue fluid pressure measured by the needle method and pancreatic duct morphology was studied in 16 patients with chronic pancreatitis. After preoperative endoscopic retrograde pancreatography (ERP) the patients were submitted to a drainage operation. The predrainage pressures were higher in the tail of the pancreas (29 mm Hg; range, 16–37 mm Hg) than in the head (18 mm Hg; range, 2–30 mm Hg; p = 0.02). The regional pressure differences were significantly greater in four patients who had previously undergone pancreatic surgery than in the 12 patients without previous surgery. A stone, total obstruction, or major stenosis in the pancreatic duct at ERP was related to a downstream pressure gradient significantly higher than found in a non-obstructed pancreatic main duct, but the relation was not uniform. Generally, there was no significant relation between pancreatic duct diameter and pressure, but in each individual patient, the regional pressure tended to be highest in the region with the largest duct diameter. In conclusion, the study shows considerable regional pressure differences in chronic pancreatitis and indicates that the intraoperative pressure measurements give important information supplementary to ERP about the pathologic process in patients with chronic pancreatitis.     

Endoscopic approach to acute pancreatitis

This review focuses on the use of endoscopic techniques in the diagnosis and management of pancreatic disorders. Endoscopic retrograde cholangiopancreatography (ERCP) has been used primarily to evaluate and treat disorders of the biliary tree. Recently, endoscopic techniques have been adapted for pancreatic sphincterotomy, stenting, stricture dilation, treatment of duct leaks, drainage of fluid collections, and stone extraction via the major and minor papillae. In patients with acute and recurrent pancreatitis, ERCP carries a higher than average risk of post-ERCP pancreatitis. This risk can be reduced with the placement of a prophylactic pancreatic stent. Magnetic resonance cholangiopancreatography (MRCP) can establish the anatomy of the biliary and pancreatic ducts, identify pancreas divisum or pancreatic ductal strictures, depict bile duct stones, and demonstrate pancreatic or biliary duct dilation. Endoscopic ultrasound (EUS) provides a safer, less invasive, and often more sensitive measure for evaluating the pancreas and biliary tree, and allows some options for therapy. In acute and recurrent pancreatitis, EUS and MRCP can be used to establish a diagnosis; ERCP can be reserved for therapy.     

Pancreatographic findings in idiopathic acute pancreatitis

Background/purpose

Despite extensive evaluation based on clinical history, biochemical tests, and noninvasive imaging studies, the cause of acute pancreatitis cannot be determined in 10 to 30% of patients, and a diagnosis of idiopathic acute pancreatitis is made. The purpose of this study was to clarify the pancreatographic findings in patients with idiopathic acute pancreatitis.

Methods

Endoscopic retrograde cholangiopancreatography (ERCP) was performed in 34 patients with idiopathic acute pancreatitis, and the pancreatographic findings were examined. Patency of the accessory pancreatic duct was examined by dye-injection endoscopic retrograde pancreatography (ERP) in 16 of the 34 patients.

Results

In 11 patients (32%), the following anatomic abnormalities of the pancreatic or biliary system were demonstrated: complete pancreas divisum (n = 5), incomplete pancreas divisum (n = 2), high confluence of pancreaticobiliary ducts (n = 2), choledochocele (n = 1), and giant periampullary diverticulum (n = 1). Pancreatographic findings were normal in 17 patients. Eleven of these patients were examined by dye-injection ERP, and all were found to have nonpatent accessory pancreatic duct.

Conclusions

Anatomic abnormality of the pancreatic or biliary system is one of the major causes of idiopathic acute pancreatitis. Closure of the accessory pancreatic duct may play a role in the development of idiopathic acute pancreatitis in patients with a normal pancreaticobiliary ductal system.     

Comparison of regional pancreatic tissue fluid pressure and endoscopic retrograde pancreatographic morphology in chronic pancreatitis

The relation between pancreatic tissue fluid pressure measured by the needle method and pancreatic duct morphology was studied in 16 patients with chronic pancreatitis. After preoperative endoscopic retrograde pancreatography (ERP) the patients were submitted to a drainage operation. The predrainage pressures were higher in the tail of the pancreas (29 mm Hg; range, 16-37 mm Hg) than in the head (18 mm Hg; range, 2-30 mm Hg; p = 0.02). The regional pressure differences were significantly greater in four patients who had previously undergone pancreatic surgery than in the 12 patients without previous surgery. A stone, total obstruction, or major stenosis in the pancreatic duct at ERP was related to a downstream pressure gradient significantly higher than found in a non-obstructed pancreatic main duct, but the relation was not uniform. Generally, there was no significant relation between pancreatic duct diameter and pressure, but in each individual patient, the regional pressure tended to be highest in the region with the largest duct diameter. In conclusion, the study shows considerable regional pressure differences in chronic pancreatitis and indicates that the intraoperative pressure measurements give important information supplementary to ERP about the pathologic process in patients with chronic pancreatitis.     

A two-year old boy with recurrent bouts of acute abdominal pain

In a small number of patients with pancreas divisum (with stenotic minor papilla) a relative obstruction to pancreatic exocrine secretory flow results in pancreatitis. We report a 2-year-old boy presenting with recurrent bouts of abdominal pain. The diagnosis of acute pancreatitis was made based on blood biochemistry results. Ultrasound, computed tomography and magnetic resonance imaging showed several abdominal pseudocysts, peritoneal exsudate and confirmed pancreatitis but initially failed to reveal the aetiology. Ascites and cysts contained pancreatic enzymes. After weeks of combined conservative and surgical treatment, a magnetic resonance cholangiopancreaticography with secretin, showed a pancreas divisum with a cyst between the ducts of Santorini and Wirsung. Based on these findings, two endoscopic papillotomies (minor and major papilla) were performed. Three years follow-up was uneventful. In a child with recurrent pancreatitis or pancreatitis with chronic recurrent abdominal pain it is crucial to search aggressively for congenital abnormalities, including pancreas divisum. Secretin-enhanced magnetic resonance cholangiopancreaticography or diffusion-weighted magnetic resonance imaging is a valuable diagnostic tool for visualizing pancreatic duct anatomy.     

Isolated Tuberculosis of the Pancreas Masquerading as a Pancreatic Mass

A 65-yr-old woman presented For evaluation of a pancreatic mass. She had been Suffring from severe constitutional symptoms for 18 months; those symptoms included weight loss, increasing fatigue, night sweats. and recurrent fever attacks up to 40°C. Later, bluish subcutaneous nodules developed on her lower limbs. Laboratory tests yielded signs of chronic inflammation and impaired glucose tolerance with elevated serum insulin and glucagon concentrations. Skin biopsy revealed lobular panniculitis. Ultrasonography and a CT scan demonstrated enlargement of the pancreas, and endo-scopic retrograde pancreaticography disclosed displacement and stenosis of the main pancreatic duct. The patient was referred for explorative laparotomy, which was highly suggestive of a malignant pancreatic tumor. However, histological examination of the resected pancreatic and peri pancreatic mass revealed tuberculous pancreatitis. This form of isolated tuberculous pancreatitis, associated with lobular panniculitis and laboratory features consistent with a tumor of the endocrine pancreas, has not been reported previously. Active tuberculosis should be a leading differential diagnosis in a patient with an enlarged pancreas when the usual diagnostic reasoning does not yield conclusive results.     

Description and rationale of a new operation for chronic pancreatitis

An operation is described that is useful in the management of patients with chronic pancreatitis and its complications. The operation features duodenal-preserving resection of the head of the pancreas combined with longitudinal pancreaticojejunostomy of the body and tail of the pancreas. The operation has application to patients with pain or complications of chronic pancreatitis with dilated ducts in the body and tail of the pancreas who have small strictured ducts and/or small pseudocysts or ducts impacted with calculi in a markedly enlarged fibrotic pancreatic head. It also has application to patients with chronic pancreatitis complicated by common duct obstruction from small pseudocysts, fibrosis, or inflammation in the head of the pancreas. With this procedure, the common duct can often be freed up from the structures compressing it within the substance of the pancreas doing away with the necessity of a separate biliary bypass. The operation also has application to patients with a previous longitudinal pancreaticojejunostomy who have recurrent or persistent pain associated with small strictured ducts in an enlarged fibrotic pancreatic head with or without common bile duct obstruction.     

Endoscopic pancreatic duct stent placement for inflammatory pancreatic diseases

The role of endoscopic therapy in the management of pancreatic diseases is continuously evolving; at present most pathological conditions of the pancreas are successfully treated by endoscopic retrograde cholangio- pancreatography （ERCP） or endoscopic ultrasound （EUS）, or both. Endoscopic placement of stents has played and still plays a major role in the treatment of chronic pancreatitis, pseudocysts, pancreas divisum, main pancreatic duct injuries, pancreatic fistulae, complications of acute pancreatitis, recurrent idiopathic pancreatitis, and in the prevention of post-ERCP pancreatitis. These stents are currently routinely placed to reduce intraductal hypertension, bypass obstructing stones, restore lumen patency in cases with dominant, symptomatic strictures, seal main pancreatic duct disruption, drain pseudocysts or fluid collections, treat symptomatic major or minor papilla sphincter stenosis, and prevent procedure-induced acute pancreatitis. The present review aims at updating and discussing techniques, indications, and results of endoscopic pancreatic duct stent placement in acute and chronic inflammatory diseases of the pancreas.