Complete obstruction of the lower common bile duct caused by autoimmune pancreatitis: is biliary reconstruction really necessary?

Recent observations suggest that an immune response is involved in the development of chronic pancreatitis. We report a case of autoimmune pancreatitis in a patient who showed complete obstruction of the lower common bile duct. A 63‐year‐old man was admitted to a local hospital, complaining of appetite loss and back pain. The patient had obstructive jaundice, and percutaneous transhepatic gallbladder drainage was performed. Fluorography through the biliary drainage catheter showed complete obstruction of the lower common bile duct. The patient had no history of alcohol consumption and no family history of pancreatic disease. Physical examination revealed an elastic hard mass palpable in the upper abdomen. Abdominal ultrasound and abdominal computed tomography (CT) scans showed enlargement of the pancreas head. While autoimmune pancreatitis was highly likely, due to the patient's high serum immunoglobulin level, the possibility of carcinoma of the pancreas and/or lower common bile duct could not be ruled out. Laparotomy was performed, and wedge biopsy samples from the pancreas head and body revealed severe chronic pancreatitis with infiltration of reactive lymphocytes, a finding which was compatible with autoimmune pancreatitis. Cholecystectomy and biliary reconstruction, using choledochojejunostomy, were performed, because the complete bile duct obstruction was considered to be irreversible, due to severe fibrosis. After the operation, prednisolone (30 mg/day) was given orally for 1 month, and the entire pancreas regressed to a normal size. Complete obstruction of the common bile duct caused by autoimmune pancreatitis has not been reported previously; this phenomenon provides an insight into autoimmune pancreatitis and provokes a controversy regarding whether biliary reconstruction is needed for the treatment of complete biliary obstruction caused by autoimmune pancreatitis.     

Involvement of the biliary system in autoimmune pancreatitis: a follow-up study.

BACKGROUND & AIMS: The aim of this study was to define the bile duct changes associated with autoimmune pancreatitis. METHODS: Eight patients with autoimmune pancreatitis were followed for a mean of 4 years. The clinical features of these patients, including extrapancreatic bile duct changes, were examined by using biochemical parameters and several imaging modalities. Pathologic features of the pancreas and liver were examined by using the biopsy specimens of 7 patients. RESULTS: Diffuse or focal narrowing of the main pancreatic duct was observed in all patients. Histologic examination of the pancreas showed lymphoplasmacyte infiltration with severe fibrosis and acinar cell depletion. In 6 patients extrapancreatic bile duct changes such as stricture of the bile duct at hilus or intrahepatic area were observed. In 2 patients abnormalities in the bile duct and pancreas were detected simultaneously at diagnosis, and changes in the bile duct were observed later in 4 patients. Lymphoplasmacyte infiltration and fibrosis were observed in the portal area of all 7 liver biopsy samples. Five of the patients with bile duct changes received steroid therapy, and the pathological changes improved. CONCLUSIONS: Extrapancreatic bile duct changes are frequently associated with autoimmune pancreatitis. Similar pathogenic mechanism might produce the biliary tract and pancreatic abnormalities in autoimmune pancreatitis resulting in a similar histopathology in the liver and pancreas and response to steroid therapy.     

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.     

New canine model of chronic pancreatitis due to chronic ischemia with incomplete pancreatic duct obstruction

A new experimental model of chronic pancreatitis was produced by a combination of chronic ischemia and incomplete obstruction of the pancreatic duct. Ischemia was induced by ligation and separation of branches flowing into the left pancreatic lobe from the splenic artery. Incomplete ductal obstruction was achieved by ligation and separation of the minor pancreatic duct and placement of a polyethylene tube in the major pancreatic duct. Macroscopic examination at 6 months after model preparation showed that the pancreas was hard, with severe inflammatory change. In the secretin test, the flow rate of pancreatic juice, amylase output and bicarbonate concentration were significantly reduced as compared with the controls. Pancreatography revealed dilatation and meandering of the major pancreatic duct and poor visualization of its secondary and tertiary bifurcations. The histopathological findings consisted of a decrease in the pancreatic parenchyma, replacement of fat, severe inflammatory cell infiltration, extensive fibrosis and tubular complexes. This model most closely resembles human chronic pancreatitis, and is a very useful instrument.     

Polysplenia syndrome with congenital agenesis of dorsal pancreas presenting as acute pancreatitis and the role of endoscopic ultrasonography in its diagnosis

A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.     

Acute pancreatitis-onset carcinoma in situ of the pancreas with focal fat replacement diagnosed using serial pancreatic-juice aspiration cytologic examination (SPACE)

A 59-year-old woman was admitted for acute pancreatitis. Abdominal computed tomography and magnetic resonance imaging revealed a swollen pancreatic parenchyma with dilatation of the main pancreatic duct (MPD) of the pancreas tail, which was separated from the normal pancreas body side by a locally atrophic part of the pancreas. Magnetic resonance cholangiopancreatography showed MPD stricture in the pancreas tail with dilatation of the upstream MPD. Endoscopic ultrasonography revealed that the MPD stricture of the pancreas tail was surrounded by a blurred and hypoechoic area. Endoscopic retrograde cholangiopancreatography was performed for serial pancreatic-juice aspiration cytologic examination (SPACE). The result indicated adenocarcinoma. Distal pancreatectomy was performed, and the histopathological examination showed high-grade PanIN (carcinoma in situ of the pancreatic duct) of the pancreas tail with atrophy and fibrosis of the pancreatic parenchyma, and local fat replacement adjacent to the lesion. The final histopathological diagnosis was carcinoma in situ of the pancreatic duct of the pancreas tail. Acute pancreatitis and local fatty change of the pancreatic parenchyma with MPD stricture are important clinical manifestations of pancreatic carcinoma in situ (PCIS) and performing SPACE in cases of MPD stricture without a recognizable mass is preferable for a diagnosis of PCIS.     

Bile exclusion from the gut exacerbates acute pancreatitis caused by pancreatic duct obstruction in rats.

Acute pancreatic duct obstruction causes hyperamylasemia and mild pancreatic inflammation. We hypothesized that bile exclusion from the gut, which stimulates pancreatic secretion, exacerbates acute pancreatitis caused by pancreatic duct obstruction. Rats were surgically prepared with gastric, duodenal, bile, and pancreatic fistula catheters and a jugular vein catheter. After a 4-day recovery, groups of rats (a) served as controls, (b) had complete pancreatic duct obstruction for 6 h, or (c) had bile excluded from the gut for 24 h and then, during the final 6 h, complete pancreatic duct obstruction. Plasma amylase was measured, and all rats were euthanized at the end of experiments. Each pancreas was excised and weighed, and portions were fixed in formalin and glutaraldehyde. In blind fashion, each pancreas was examined under light microscopy and assigned a pancreatitis score based on presence of edema and severity of acinar cell changes and inflammation. Acute pancreatic duct obstruction was associated with increased pancreas weight, hyperamylasemia, and elevated pancreatitis score; moderate acinar cell vacuoles, which were observed in the cytoplasm near the basolateral membrane, and loss of microvilli were noted with electron microscopy. Bile exclusion from the gut exacerbated the acute pancreatitis caused by pancreatic duct obstruction; acinar cell distortion and destruction, as well as marked inflammation, were seen microscopically. These observations suggest that the absence of intestinal bile contributes to the pathogenesis of acute pancreatitis associated with pancreatic duct obstruction.     

Pancreatic carcinoma accompanied by pseudocyst: Report of two cases

Two cases of pancreatic cancer accompanied by pseudocyst are reported. Case 1 was a 60-year-old man who was admitted to our hospital complaining of left lower abdominal discomfort. A cystic lesion, about 3 cm in diameter, was found in the pancreatic tail by ultrasonography (US) and computed tomography (CT). No signs of chronic pancreatitis were found. At operation, an elastic, hard, white tumor, about 1 cm in diameter, was felt adjacent to the cystic lesion on the duodenal side. Histologically, this tumor was a duct cell carcinoma with an adjacent pseudocyst upstream of the pancreas. Case 2 was a 57-year-old man who complained of back pain and loss of body weight. US and CT examination revealed a cystic lesion, 11×7 cm in size, in the tail of the pancreas. Histological examination of the resected speciment revealed both a duct cell carcinoma, 3 cm in size, in the body of the pancreas and a pseudocyst, 9 cm in size. Pseudocysts accompanying carcinoma are thought to develop from obstruction of the pancreatic duct by the carcinoma, followed by intraductal high pressure and disruption of ductules upstream of the pancreas. Thus, we should pay careful attention to pseudocyst of the pancreas, especially when signs of diffuse chronic inflammation cannot be found, to help identify duct cell carcinoma in the early stage. Further detailed examinations of the cyst fluid or pancreatic juice, such as cytology, tumor marker determinations, or establishment of K-ras codon 12 mutation, are needed.     

Ultrasound secretin test in patients with pancreas divisum--an aid in the diagnosis of papillary or dorsal duct stenosis?

BACKGROUND: Pancreas divisum is a common anatomical variant of pancreatic ductal anatomy. Obstruction of the accessory papilla could cause pain and pancreatitis. It has been suggested that accessory papillary sphincter obstruction can be assessed by sonographic measurement of pancreatic duct diameter after secretin stimulation. METHODS: We now compared our results of sonographic pancreatic duct diameter measurements before and during 10 min after intravenous injection of 1 CU secretin per kg body weight in 32 patients with confirmed pancreas divisum and 20 healthy volunteers. RESULTS: The healthy controls showed a short-lasting duct caliber enlargement by about 93% of the basal diameter within 5 min after secretin injection. 25 pancreas divisum patients without pancreatic disease had a secretin-induced duct dilatation by about only 58%. In four patients with pancreas divisum and chronic pancreatitis no or just a slight duct dilatation was observed after stimulation. Two patients with dorsal duct stenosis as well as one patient with accessory papilla stenosis, however, showed a marked and prolonged secretin-induced duct enlargement by about 155% of the basal duct diameter. CONCLUSION: In this investigation pancreatic duct response to secretin stimulation in pancreas divisum patients without pancreatic disease was less marked than in normal individuals. Thus, a particularly distinct and long-lasting duct dilatation could support the suspicion of accessory papilla or pancreatic duct stenosis.     

Chronic obstructive pancreatitis,nesidioblastosis, and small endocrine pancreatic tumor

A patient is described in whom a small 0.8-mm-diameter endocrine tumor was found in association with localized pancreatitis, stricture of the main pancreatic duct, and nesidioblastosis. The endocrine tumor, localized 7 mm from the main duct, obstructed a collateral pancreatic duct and four of its branches. Pancreatitis was localized into two areas. It was fairly severe in a small area of the body drained by the obstructed collateral duct and extended around it, reaching the main pancreatic duct which was partly occluded at this level by periductal fibrosis. Pancreatitis was mild in the left part of the pancreas distally from the incomplete stricture of the main duct. The head and body of the pancreas between the stricture and the duodenum were normal. Nesidioblastosis was present in the obstructed parts of the pancreas. It is suggested that the tumor could have caused pancreatitis, stenosis of the main duct, and nesidioblastosis.     

A case of colon obstruction developed as a complication of acute pancreatitis]

In acute pancreatitis, colonic complications such as mechanical obstruction, ischemic necrosis, hemorrhage, and fistula are rare but their outcomes are fatal. It is known that colonic obstruction in acute pancreatitis is more likely found in splenic flexure and transverse colon caused by severe inflammation of body and tail of pancreas leading to pressure necrosis. A 43-year-old man presented with abdominal distension lasting for 2 weeks. The patient had been admitted to our institution 6 weeks prior to the current admission, and the abdominal CT scan performed during the first admission revealed the pancreatic enlargement with peri-pancreatic fatty infiltration and fluid collection. At that time he was diagnosed as acute pancreatitis. The conservative management resulted in clinical improvement so that the patient was discharged. Upon the second admission, abdominal CT scan revealed multiple pseudocysts in the tail portion of pancreas with concominant wall thickening and narrowing of the proximal descending colon, and a dilatation of the bowel proximal to the splenic flexure. An obstruction of the descending colon as a complication of acute pancreatitis was suspected and the patient underwent left hemicolectomy. Abdominal distension was relieved after the operation and he was discharged on the 15th hospital days.     

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.     

A giant retention cyst of the pancreas (cystic dilatation of dorsal pancreatic duct) associated with pancreas divisum

We describe a rare case of pancreas divisum associated with a giant retention cyst (cystic dilatation of the dorsal pancreatic duct), presumably formed following obstruction of the minor papilla. The patient was treated by pancreatico(cysto)jejunostomy. A 50-year-old man was admitted with complaints of increasing upper abdominal distension and body weight loss. There was no previous history of pancreatitis, gallstones, drinking, or abdominal injury. An elastic-hard tumor-like resistance was palpable in the upper abdomen. Computed tomography and ultrasound (US) examinations revealed a giant cystic lesion expanding from the pancreas head to the tail. Endoscopic retrograde cholangiopancreatography findings showed a looping pancreatic duct which drained only the head and uncinate process of the pancreas to the main papilla. A US-guided puncture to the cystic lesion revealed that the lesion continued to the main pancreatic duct in the tail of pancreas. The lesion was connected to a small cystic lesion, which was located inside the minor papilla, and ended there. The amylase level in liquid aspirated from the cyst was 37 869 IU/l, and the result of cytological examination of the liquid showed class II. A pancreatico(cysto)jejunostomy was performed, with the diagnosis being pancreas divisum associated with a retention cyst following obstruction of the minor papilla. The histological findings of a specimen from the cyst wall revealed that the wall was a pancreatic duct covered with mildly inflammatory duct epithelium; there was no evidence of neoplasm. The patient is currently well, and a CT examination 2 years after the operation showed disappearance of the cyst and normal appearance of the whole pancreas. Received: April 24, 2001 / Accepted: September 14, 2001     

Secretin-stimulated magnetic resonance pancreaticogram to assess pancreatic duct outflow obstruction in evaluation of idiopathic acute recurrent pancreatitis: a pilot study

Magnetic resonance pancreatography is a new modality to visualize the pancreatic duct. Prolonged dilation of the pancreatic duct following secretin administration may suggest obstruction at the level of the pancreatic duct orifice. We describe 10 patients with idiopathic acute recurrent pancreatitis who underwent secretin-stimulated magnetic resonance pancreatography with subsequent endoscopic retrograde pancreatogram with or without manometry. All patients had complete visualization of the main pancreatic duct and no evidence of chronic duct disease. Two patients had pancreas divisum. Three had prolonged dilation of the pancreatic duct on secretin-stimulated magnetic resonance pancreatography and evidence of pancreatic duct outflow obstruction. Four additional patients with pancreatic duct outflow obstruction had normal secretin-stimulated magnetic resonance pancreatography. In conclusion, secretin stimulated magnetic resonance pancreatography provides high quality pancreatic duct images and has high specificity but low sensitivity for diagnosing pancreatic duct outflow obstruction using manometric/clinical criteria.     

Minute pancreatic carcinoma with initial symptom of acute pancreatitis

We experienced a case of minute pancreatic carcinoma in a 59‐year‐old man who complained of upper abdominal pain after drinking alcohol. Abdominal ultrasonography (US) revealed dilatation of the main pancreatic duct (MPD). Abdominal computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) showed slight dilatation of the MPD and its obstruction near the portal vein. Endoscopic retrograde cholangiopancreatography (ERCP) demonstrated occlusion of the MPD, and cytology of aspirated pancreatic juice was negative for malignancy. With the diagnosis of benign localized obstruction of the MPD, the patient underwent surgery. There was a clear demarcation of hardness and color of the pancreas on the left margin of the superior mesenteric vein, and the caudal pancreas was hard and fibrotic. Intraoperative US revealed slight dilatation of the MPD, and the aspiration cytology result was class IV. First, segmental resection of the pancreas was performed, but pathological examination of frozen section showed neither malignancy nor stenotic lesion. An additional small portion of the proximal pancreas was resected. The specimen included a ductal carcinoma, 5mm in diameter. Accordingly, a pylorus‐preserving pancreatoduodenectomy was performed. Microscopically, the minute carcinoma had already penetrated the duct wall and infiltrated lymph vessels and veins. The patient has been under close observation at our outpatient clinic, and so far there have been no signs of recurrence. To improve the poor prognosis of pancreatic cancer, we should be alert to the occurrence of acute pancreatitis as an initial symptom.     

Pancreatic stone predominantly composed of fatty acid calcium.

A 41-year-old man with chronic pancreatitis and pancreatic stones predominantly composed of fatty acid calcium is reported. He had complained of occasional abdominal pain for 10 years and visited the hospital because of a severe attack of abdominal pain. Laboratory data supported a diagnosis of pancreatitis. Computed tomography (CT) showed a high-density area in the head of the pancreas, and the CT number of this high-density area was lower than usual for pancreatic stones. Ultrasonography and endoscopic retrograde pancreatography showed a cystic lesion with small pancreatic stones in the head of the pancreas and irregular dilatation of the main pancreatic duct. Pancreaticojejunostomy and resection of pancreatic cyst were carried out for repeated episodes of abdominal pain under the diagnosis of chronic pancreatitis. The pancreatic stones obtained at surgery were proved to be mainly composed of fatty acid calcium after analysis of chemical composition of the stones. Fatty acid calcium was sometimes found in the biliary stones but never in the pancreatic stones.     

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.     

Obstructing Pseudocyst of the Duct of Santorini in Pancreas Divisum

Pancreas divisum is a pancreatic duct anomaly that occurs due to failure of fusion of the dorsal and ventral ducts. While recognition of this anomaly is increasing due to more aggressive endoscopic retrograde cholangiopancreatography, its significance remains unclear. A patient with chronic pancreatitis and a history of alcohol abuse was noted to have pancreas divisum. At surgical exploration, intraoperative pancreatography revealed an obstructing pseudocyst of the duct of Santorini. Extended sphincteroplasty and cystduodenostomy as well as Roux-en-Y pancreatojejunostomy were necessary to insure adequate accessory duct drainage. Surgical therapy of pancreas divisum in chronic pancreatitis should be designed to correct existing pancreatic duct obstruction.     

Annular pancreas associated with carcinoma in the dorsal part of pancreas divisum

Summary A carcinoma in the dorsal part of the pancreas divisum with an annular pancreas in the anterior part is reported. A 79-yr-old female was admitted in our hospital complaining of epigastralgia. Computed tomography (CT) and ultrasound (US) showed an irregular mass in the pancreatic body. A pancreatogram obtained through the major duodenal papilla demonstrated only the ventral pancreatic duct that encircled the duodenum. Contrast medium injected from the minor duodenal papilla showed Santorini’s duct obstruction at the neck portion of the pancreas without communication with the ventral pancreatic duct. The patient died with liver metastases. Autopsy confirmed annular pancreas and a 6-cm tumor in the pancreatic body extending to the pancreatic head and pancreas divisum. Pancreatic carcinoma; histologically a moderately differentiated adenocarcinoma; originated from the dorsal part of pancreas divisum. To our knowledge this is the first report of pancreatic carcinoma associated with annular pancreas coexistent with pancreas divisum.     

Autoimmune pancreatitis associated with retroperitoneal fibrosis

CONTEXT: Autoimmune pancreatitis is sometimes associated with other autoimmune diseases. We have presented two cases of autoimmune pancreatitis with retroperitoneal fibrosis and compared our findings with those found in the literature. CASE 1: A 71-year-old male developed anorexia and weight loss. Abdominal ultrasonography (US) and computed tomography (CT) showed diffuse swelling of the pancreas and the peritoneal soft tissue surrounding the aorta, associated with right hydronephrosis. Endoscopic retrograde pancreatography showed narrowing of the main pancreatic duct. He was diagnosed as having autoimmune pancreatitis associated with retroperitoneal fibrosis and underwent steroid therapy. After 3 weeks, a follow-up CT showed a marked reduction in the size of both the pancreas and retroperitoneal mass. CASE 2: A 62-year-old male was admitted to another hospital complaining of obstructive jaundice. Abdominal CT and US showed swelling of the pancreas. Endoscopic retrograde cholangiopancreatography demonstrated stenosis of the lower bile duct and narrowing of the main pancreatic duct. With the diagnosis of pancreatic head carcinoma, a choledochojejunostomy and a gastrojejunostomy were performed. Histological examination of the biopsy of the pancreatic mass revealed marked fibrosis with lymphoplasmacytic infiltration. One year later, a retroperitoneal mass was detected on follow-up CT. He was treated with prednisolone for two years. Recurrence of retroperitoneal mass with left hydronephrosis occurred 18 months later. There was no sign of recurrence of the autoimmune pancreatitis. He was again treated with prednisolone, and the retroperitoneal mass was gradually reduced. CONCLUSIONS: A total of 7 cases including the present cases have been reported. All were middle-aged males. Steroid therapy was effective for both the pancreatic and the retroperitoneal masses.