Resection of carcinoma in situ and minimally invasive carcinoma of the pancreas in a patient presenting with stenosis and post-stenotic dilatation of the main pancreatic duct on endoscopic ultrasonography and positive serial pancreatic juice aspiration cytology.

We report a rare case of a 67-year-old man who underwent resection of carcinoma in situ and minimally invasive carcinoma of the pancreas. The patient presented with upper abdominal and back pain. No definite pancreatic mass was detected on abdominal ultrasonography, computed tomography, magnetic resonance imaging (MRI), or endoscopic ultrasonography (EUS). However, EUS and MRI demonstrated stenosis of the main pancreatic duct (MPD) in the body and post-stenotic dilatation, resulting in mild dilatation of MPD in the tail. Serial pancreatic juice aspiration cytology after endoscopic nasopancreatic drainage was suggestive of pancreatic adenocarcinoma. Examination of the distal pancreatectomy specimen demonstrated carcinoma in situ in MPD and branches, with multiple intraepithelial neoplastic lesions in the background pancreas and an additional focus of minimally invasive carcinoma.

Recurrence of renal cell carcinoma diagnosed using contralateral adrenal biopsy with endoscopic ultrasound-guided fine-needle aspiration.

A 76-year-old female in whom a renal cell carcinoma (RCC) lesion was resected 19 years previously presented to our hospital with cognitive dysfunction. Magnetic resonance imaging and computed tomography revealed nodules in the brain, lung, adrenal gland and a pelvic osteolytic lesion. To identify the primary cancer site, the present study performed endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) of the left adrenal lesion. Consequently, the pathological findings of the tissue obtained by EUS-FNA were similar to those of the previous nephrectomy specimen, revealing that the adrenal lesion was the recurrence of RCC. The majority of the metastatic lesions in the patient were reduced in size by the multiple kinase inhibitor, pazopanib. Contralateral adrenal metastasis of RCC is rare and the use of EUS-FNA in the diagnosis of adrenal lesions remains to be elucidated. This is a rare case of adrenal lesion, diagnosed by EUS-FNA. Therefore, EUS-FNA is considered to be a useful diagnostic modality of adrenal metastases from unidentified primary tumor types.

Expression of Akt kinase-interacting protein 1, a scaffold protein of the PI3K/PDK1/Akt pathway, in pancreatic cancer.

OBJECTIVES: Akt kinase-interacting protein 1 (Aki1) has been reported to be a scaffold protein of the PI3K (phosphoinositide 3-kinase)/PDK1 (3-phosphoinositide-dependent protein kinase)/Akt pathway and to interact with epidermal growth factor receptor signaling. Although Aki1 has been reported to be expressed in lung cancer, the significance of its expression in pancreatic cancer has not been clarified. METHODS: The expression of Aki1 and its associated proteins was assayed in pancreatic cancer cell lines, and its involvement in cell viability was examined by treatment with Aki1 small interfering RNA. We also assessed the immunohistochemical expression of Aki1 in tissue samples from 60 patients with pancreatic cancer. RESULTS: All of the pancreatic cancer cell lines expressed Aki1 and its associated proteins at various levels. Treatment with Aki1 small interfering RNA or PI3K inhibitor inhibited the viability of Panc1 cells. Silencing of Aki1 in Panc1 cells reduced the phosphorylation of Akt and increased the phosphorylation of cleaved PARP. The Aki1 was expressed in 25 (42%) of the 60 pancreatic cancers, but there was no correlation between Aki1 expression and clinicopathologic parameters. We observed a statistically significant correlation between Aki1 and p-Akt expression (P = 0.016). CONCLUSIONS: Inhibition of the Aki1-Akt axis may be a therapeutic target in some patients with pancreatic cancer.

Synchronous triple cancers of the pancreas, stomach, and cecum treated with S-1 followed by pancrelipase treatment of pancreatic exocrine insufficiency.

CONTEXT: Pancreatic cancer is frequently complicated by malignancies in other organs. However, synchronous triple cancers including pancreatic cancer have been seldom reported in the English language literature. CASE REPORT: We describe the rare case of a 77-year-old man with triple cancers of the pancreas, stomach, and cecum. Biopsies revealed that all three tumors were adenocarcinomas. The pancreatic and gastric tumors were positive for cytokeratin 7 and negative for cytokeratin 20, whereas the cecal tumor was negative for cytokeratin 7 and positive for cytokeratin 20. K-ras mutations were present at codon 12 in the pancreatic tumor and at codon 13 in the cecal tumor, but were absent from the gastric tumor. Since the three tumors had different characteristics, the patient was diagnosed with synchronous triple cancers. Because invasive surgery was required to remove all three tumors and the patient had risk factors for surgery, we elected to treat him with chemotherapy. All three cancers were markedly reduced in size by treatment with cycles of 100 mg/day S-1 for 2 weeks, followed by a 1-week rest. The patient later developed hypoproteinemia and anasarca, which was diagnosed as pancreatic exocrine insufficiency due to pancreatic head cancer. Treatment with pancrelipase resulted in dramatic improvements in hypoproteinemia and anasarca. CONCLUSIONS: This is the first case report in which S-1 was effective in triple cancers of the pancreas, stomach, and cecum. Patients with pancreatic head cancer should be monitored for pancreatic exocrine insufficiency.

Successful treatment of unresectable gallbladder cancer with low-dose paclitaxel as palliative chemotherapy after failure of gemcitabine and oral S-1: A case report.

A 56-year-old female with metastatic gallbladder cancer involving the liver and stenosis of the hilar bile duct was treated with gemcitabine (1,000 mg/m(2)) plus S-1 (60 mg/m(2)). After 9 cycles of therapy, CT showed evidence of stable disease; however, the serum CEA level was increased. Therefore, the chemotherapy regimen was changed to weekly low-dose paclitaxel (60 mg/m(2)). After 12 cycles of therapy, paclitaxel was reduced to 30 mg/m(2) as the patient developed neutropenia. The patient completed 32 cycles of therapy, and the tumor was reduced in size and marked improvement in bile duct stenosis was noted without any impairment in quality of life. The patient succumbed to the disease 25 months after treatment was initiated. Thus, in this case paclitaxel was more effective than gemcitabine plus S-1. Palliative chemotherapy with paclitaxel after failure of gemcitabine and 5-FU was well-tolerated; therefore, it may be an effective treatment for biliary tract cancer (BTC). A phase I study of palliative chemotherapy with weekly low-dose paclitaxel following gemcitabine (plus cisplatin) and 5-FU is currently in progress in patients with unresectable or recurrent BTC.

[A case of poorly-differentiated neuroendocrine carcinoma of the ascending colon with multiple liver metastases successfully treated with cisplatin and irinotecan].

A 74-year-old woman diagnosed with poorly-differentiated neuroendocrine carcinoma originating from the ascending colon was referred to our hospital. She had felt anorexia, abdominal pains and her (ECOG) performance status was 3. Her CT scan showed that some abdominal lymph nodes were swelling and that there were many metastatic lesions occupying most of the liver. We started chemotherapy with cisplatin and irinotecan according to a regimen for small cell lung cancer. Considering her poor PS, both of the drugs were administered at 30mg/m² twice 4 weeks in the first course of chemotherapy. Her anorexia and abdominal pains immediately disappeared, and CT scan showed that all of the metastases were decreased in size. After 4 courses, however, some of the metastatic lesions were increased in size. She died 8 months after diagnosis. The tumor marker doubling time was 17 days.

[Cancer of unknown primary site with epidermal growth factor receptor mutation for which gefitinib proved effective].

We report a 53-year-old man with cancer of an unknown primary site with an epidermal growth factor receptor mutation for which gefitinib was effective. In 2007, he complained of left gluteal pain and right cervical lymph node swelling. He was given a diagnosis of adenocarcinoma at the biopsy of the right cervical lymph node. Although metastases of multiple lymph nodes, bone, and bilateral adrenal glands were found, the primary site could not be determined on close examination, resulting in a diagnosis of cancer of unknown primary site(poor prognosis group). He was then treated with systemic chemotherapy. After he showed resistance to chemotherapy, he received gefitinib as third-line therapy because the tumor harbored an epidermal growth factor receptor(EGFR)mutation. Subsequently, multiple metastatic tumors gradually reduced and clinical benefit was observed for a long time.

Endoscopic findings of upper gastrointestinal lesions in patients with pancreatic cancer.

CONTEXT: Pancreatic cancer is often complicated with upper gastrointestinal lesions. However, there have been few endoscopic studies in pancreatic cancer patients. We retrospectively investigated the upper gastrointestinal lesions in patients with pancreatic cancer who underwent upper gastrointestinal endoscopy. METHODS: Upper gastrointestinal endoscopy was performed in 75 patients with pancreatic cancer between 2003 and 2010. We examined upper gastrointestinal lesions, such as gastroduodenal invasion, ulcers, esophagogastric varices, radiation-induced gastroduodenal mucosal lesions, and portal hypertensive gastropathy. RESULTS: Among the 53 patients with pancreatic cancer who underwent upper gastrointestinal endoscopy at diagnosis, 23 gastrointestinal lesions were observed in 20 patients (38%) as follows: gastroduodenal invasion (n=11), esophagogastric varices (n=7), gastroduodenal ulcers (n=3), portal hypertensive gastropathy (n=1) and duodenal metastasis (n=1). Among the 75 patients with pancreatic cancer, 56 gastrointestinal lesions were identified in 46 patients (61%) during the clinical course as follows: gastroduodenal invasion (n=20), esophagogastric varices (n=14), radiation-induced gastroduodenal mucosal lesions (n=9), gastroduodenal ulcers (except radiation-induced ulcers) (n=8), portal hypertensive gastropathy (n=3), duodenal metastasis (n=1), and gastrointestinal bleeding from unknown primary site (n=1). Twenty-nine (52%) of the 56 gastrointestinal lesions showed symptoms related to the lesions. Fifteen (27%) lesions were accompanied by upper gastrointestinal bleeding. Fourteen (25%) lesions developed according to the progression of pancreatic cancer. CONCLUSION: We should pay attention to upper gastrointestinal lesions in patients with pancreatic cancer.

Randomized phase II study of gemcitabine and S-1 combination versus gemcitabine alone in the treatment of unresectable advanced pancreatic cancer (Japan Clinical Cancer Research Organization PC-01 study).

PURPOSE: To evaluate the efficacy and safety of the combination of gemcitabine (GEM) and S-1 (GS) in comparison to GEM alone (G) for unresectable pancreatic cancer. METHODS: In this multicenter randomized phase II study, we randomly assigned unresectable pancreatic cancer patients to either the GS group or the G group. The GS group regimen consists of intravenous 1,000 mg/m(2) GEM during 30 min on days 1 and 8, combined with 80 mg/m(2) oral S-1 twice daily on days 1-14, repeated every 3 weeks. On the other hand, the G group regimen consists of intravenous 1,000 mg/m(2) GEM on days 1, 8, and 15, repeated every 4 weeks. The primary endpoint was objective response rate (ORR). Secondary end points included treatment toxicity, clinical response benefit, progression-free survival (PFS), and overall survival. RESULTS: We registered 117 patients from 16 institutions between June 2007 and August, 2010. The ORR of the GS group was 28.3%, whereas that of the G group was 6.8%. This difference was statistically significant (P = 0.005). The disease control rate was 64.2% in the GS group and 44.1% in the G group. Median PFS was 6.15 months in the GS group and 3.78 month in the G group. This was also statistically significant (P = 0.0007). Moreover, the median overall survival (OS) of the GS group was significantly longer than that of the G group (13.7 months vs. 8.0 months; P = 0.035). The major grade 3-4 adverse events were neutropenia (54.7% in the GS group and 22.0% in the G group), thrombocytopenia (15.1% in the GS group and 5.1% in the G group), and skin rash (9.4% in the GS group). CONCLUSIONS: The GS group showed stronger anticancer activity than the G group, suggesting the need for a large randomized phase III study to confirm GS advantages in a specific subset.

Multiple factors influencing the release of hTERT mRNA from pancreatic cancer cell lines in in vitro culture.

Since telomerase expression is highly prevalent in human cancers, the quantitation of serum/plasma hTERT (human telomerase reverse transcriptase) mRNA levels may be useful for early detection of PCa (pancreatic cancer). To analyse the correspondence between exhTERT (extracellular hTERT) mRNA levels and hTERT expression, we designed a cell culture system to investigate factors modulating the extracellular levels of hTERT mRNA in media conditioned by eight PCa cell lines. We found that the level of exhTERT mRNA was dependent on cell growth rate. MIAPaCa-2, PANC-1, KLM-1 and PK-9 cells expressed high levels of exhTERT mRNA, independent of cell density, whereas proliferating PK-59, BxPC-3 and PK-45H cells released low levels of exhTERT mRNA. The augmented release of mRNA by spontaneous dead MIAPaCa-2 cells was further increased at postconfluence. In Capan-1 cells, low correspondence of marker was also due to RNase secretion. Upon reaching confluence, some PCa cell lines showed down-regulation of hTERT expression. Following cell-cell adhesion, as shown by E-cadherin engagement, PK-59 cells showed levels of extracellular message below the limits of detection, a loss not due to an increase in message degradation. These results suggest that the levels of exhTERT mRNA in the medium of PCa cell lines are altered not only in response to cell growth rate and cell destruction, but are responsive to extracellular cues such as RNases and cell density. A cell-free assay for exhTERT mRNA may therefore not be useful for early detection of PCa.

The EGFR ligands amphiregulin and heparin-binding egf-like growth factor promote peritoneal carcinomatosis in CXCR4-expressing gastric cancer.

PURPOSE: Peritoneal carcinomatosis, often associated with malignant ascites, is the most frequent cause of death in patients with advanced gastric cancer. We previously showed that the CXCR4/CXCL12 axis is involved in the development of peritoneal carcinomatosis from gastric cancer. Here, we investigated whether epidermal growth factor receptor (EGFR) ligands are also involved in the development of peritoneal carcinomatosis from gastric cancer. EXPERIMENTAL DESIGN: The functional involvement of expression of the ErbB family of receptors and/or EGFR ligands was examined in CXCR4-expressing human gastric cancer cells and fibroblasts, clinical samples (primary tumors and ascites), and an animal model. RESULTS: High concentration of the EGFR ligands amphiregulin and heparin-binding EGF-like growth factor (HB-EGF), as well as of CXCL12, were present in malignant ascites. Human gastric cancer cell lines and primary gastric tumors, with high potential to generate peritoneal carcinomatosis, expressed high levels of EGFR and CXCR4 mRNA and protein. Both amphiregulin and HB-EGF enhanced the proliferation, migration, and functional CXCR4 expression in highly CXCR4-expressing gastric cancer NUGC4 cells. Amphiregulin strongly enhanced the proliferation of NUGC4 cells, whereas HB-EGF markedly induced the migration of fibroblasts. Moreover, HB-EGF and CXCL12 together enhanced TNFα-converting enzyme (TACE)-dependent amphiregulin shedding from NUGC4 cells. In an experimental peritoneal carcinomatosis model in mice, cetuximab effectively reduced tumor growth and ascites formation. CONCLUSIONS: Our results strongly suggest that the EGFR ligands amphiregulin and HB-EGF play an important role, interacting with the CXCL12/CXCR4 axis, in the development of peritoneal carcinomatosis from gastric cancer, indicating that these two axes may be potential therapeutic targets for peritoneal carcinomatosis of gastric carcinoma.

[PI3K inhibitor].

PI3K-Akt is an important signal transduction pathway which acts downstream from various growth factor receptors. The PI3K-Akt pathway is activated in many types of cancers by several mechanisms, including growth factor receptor activation, loss of PTEN, and PI3K gene mutations. Therefore, PI3K is thought to be an ideal therapeutic target. A large number of PI3K inhibitors are being developed and evaluated in clinical trials.

[Combined chemotherapy with cisplatin plus vinorelbine showed efficacy in a case of metastatic primary cutaneous adenoid cystic carcinoma].

We report a case of a 65-year-old man with metastatic primary cutaneous adenoid cystic carcinoma that was effectively treated by combination chemotherapy with cisplatin (CDDP) plus vinorelbine(VNR). He detected a tumor mass on the anterior surface of his left patella in 2003 and underwent a tumorectomy in October 2008. He was given a diagnosis of primary cutaneous adenoid cystic carcinoma with metastatic lesions to multiple lungs and left tibial bone and then given chemotherapy combining CDDP plus VNR as the first treatment in December 2008. By this treatment for six cycles, the lung metastatic tumors gradually reduced on chest CT. We reported the efficacy of combined treatment with CDDP plus VNR for primary cutaneous adenoid cystic carcinoma, because this clinical condition was very rare and the standard treatment has still not been established.

Metastatic renal cell carcinoma complicated with diffuse alveolar hemorrhage: a rare adverse effect of sunitinib.

We report the case of a 67-year-old man with metastatic papillary renal cell carcinoma (RCC) who developed bloody sputum after the administration of sunitinib. Chest computed tomography revealed diffuse ground-glass opacity lesions, and bloody bronchoalveolar lavage fluid was obtained by flexible bronchoscopy. The abnormal shadows promptly regressed after withdrawal of sunitinib. In four cycles of sunitinib treatment, he suffered from controllable diffuse alveolar hemorrhage. Finally, he died of respiratory failure 8 months after onset. This is the first case report of diffuse alveolar hemorrhage as an adverse effect of sunitinib in metastatic papillary RCC. Care should be taken with pulmonary hemorrhage in the use of anti-angiogenesis agents in not only squamous cell lung cancer, but also metastatic lung tumors.

Combined chemotherapy with carboplatin plus irinotecan showed favorable efficacy in a patient with relapsed small cell carcinoma of the prostate complicated with meningeal carcinomatosis.

We report the case of a 65-year-old man with recurrent prostate cancer who presented with meningeal carcinomatosis. In September 2007, he had been diagnosed with mixed type small cell carcinoma and adenocarcinoma at clinical stage T4N1M1 (primary prostate tumor with multiple bone, liver, and lymph node metastases) and hormonal therapy had been administered. Following an increase in the level of pro-gastrin-releasing peptide (ProGRP), combined chemotherapy with cisplatin plus etoposide was implemented and showed efficacy in targeting the small cell carcinoma. In March 2008, he presented with signs of meningeal irritation; his condition deteriorated quickly and multiple brain metastases were confirmed by magnetic resonance imaging (MRI). A sample of cerebrospinal fluid collected by lumbar puncture showed cancer cells and an elevated level of ProGRP. Small cell carcinoma of the prostate complicated with meningeal carcinomatosis was diagnosed. A different chemotherapy regimen was then administered, consisting of a combination of carboplatin plus irinotecan, which is one of the most common first-line treatments for extensive-stage small cell lung carcinoma. From day 20 after the initiation of this therapy, he gradually recovered from the signs of meningeal irritation, and brain MRI showed nearly normal findings; also, the serum level of ProGRP was reduced. In conclusion, we report the efficacy of combined treatment with carboplatin plus irinotecan for small cell carcinoma of the prostate complicated with meningeal carcinomatosis. Because this clinical condition is extremely rare, a gold standard treatment has yet to be established.

Advanced hepatocellular carcinoma treated effectively with irinotecan via hepatic arterial infusion followed by proton beam therapy.

We report a 48-year-old man with hepatocellular carcinoma (HCC) treated with hepatic arterial infusion (HAI) chemotherapy followed by proton beam therapy. The HCC lesion in this patient was 88 mm in diameter, with portal vein tumor thrombosis in the right lobe of the liver. He was first treated with 5-fluorouracil, cisplatin, and isovorin, administered by HAI, combined with interferon-alpha, and he was subsequently treated with epirubicin and mitomycin-C administered by HAI. However, no definite efficacy of either of these treatments was observed. Then, after 3 weeks' continuous administration of irinotecan by HAI, the tumor size decreased to 68 mm in diameter. However, 3 months after reduction of the tumor, the tumor had become enlarged to 100 mm in diameter and intrahepatic metastases were prominent. Angiographic findings indicated that the HCC was fed not only from the right hepatic artery but also from the left gastric and right and left subphrenic arteries. After rearrangement of the arteries, and 3 months' continuous HAI chemotherapy with irinotecan, plus hyperthermia, the tumor size had decreased to 50 mm in diameter. The reduction rate of the main tumor according to the Response Evaluation Criteria in Solid Tumors was 43%; therefore, the efficacy of this treatment was judged as a partial response. Two months after reduction of the tumor, the patient's serum alpha-fetoprotein (AFP) level was elevated, and so docetaxel was administered by HAI instead of irinotecan. The liver tumors showed gradual enlargement during the administration of docetaxel, although the AFP level was suppressed. Proton beam therapy was instituted and the liver tumors showed necrosis after this therapy. The patient died of hepatic failure and distant metastases 6 years after the onset of HCC. As far as we know, this is the first case report of HCC treated effectively with irinotecan administered by HAI followed by proton beam therapy in which tumor suppression and the long-term survival of the patient were observed.

Cancer of unknown primary site in which tumor marker-oriented chemotherapy was effective and pancreatic cancer was finally confirmed at autopsy.

We report a 47-year-old man with cancer of unknown primary site in whom pancreatic cancer was confirmed at autopsy. Although a primary lesion was not confirmed, we planned to perform tumor marker-oriented chemotherapy because pancreatic cancer was suspected as the primary lesion based on tumor markers and pathological findings from metastatic lymph node. Neither S-1 nor gemcitabine was effective. However, gemcitabine combined with low-dose cisplatin therapy resulted in a marked decrease in the size of tumors. Microscopic examination at autopsy revealed poorly differentiated adenocarcinoma in the pancreatic head, although a pancreatic mass was not clear macroscopically.

[An autopsy case of pancreatic cancer with poor performance status due to peritonitis carcinomatosa treated successfully with continuous infusion of low-dose 5-FU with octreotide].

A 60-year-old man with pancreatic cancer was admitted due to massive ascites in the course of gemcitabine treatment. Cachexic condition progressed due to peritonitis carcinomatosa. Continuous infusion of low dose 5-FU with octreotide was carefully started. Almost all of ascites disappeared after 4 courses of treatment and his general condition markedly improved. This patient died of pneumonia about 13 months after diagnosis of peritonitis carcinomatosa. Autopsy was undergone, and the effect of chemotherapy was confirmed.

A case of pancreatic cancer with formation of a mass mimicking alcoholic or autoimmune pancreatitis in a young man. Possibility of diagnosis by hypermethylation of pure pancreatic juice.

CONTEXT: Mass-forming pancreatitis can be divided into two distinct types: alcoholic and autoimmune. There have been some cases of an ambiguous diagnosis although care was taken to differentiate between alcoholic mass-forming pancreatitis, focal type autoimmune pancreatitis and pancreatic cancer. CASE REPORT: We report a case of pancreatic cancer mimicking alcoholic or autoimmune pancreatitis with the formation of a mass in a 32-year-old man with a history of heavy drinking. Although both serum immunoglobulin G and immunoglobulin G4 levels were normal, many serum auto-antibodies, including the antinuclear antibody, were detected. After he stopped drinking, abdominal computed tomography showed a pancreatic head mass 28 mm in diameter with little and weak enhancement in the early and delayed phases, respectively. Endoscopic retrograde cholangiopancreatography showed an obstruction of the main pancreatic duct in the pancreatic head and marked stenosis of the lower common bile duct. Although a percutaneous ultrasound-guided pancreatic biopsy demonstrated no evidence of autoimmune pancreatitis, he was treated with prednisolone to test the efficacy of steroid therapy. However, the pancreatic mass became enlarged after steroid therapy, and he underwent surgery during which the mass was found to be pancreatic cancer. Although the patient was treated with gemcitabine, he died 5 months after surgery. We retrospectively assessed DNA hypermethylation in the patient's pure pancreatic juice obtained on admission. We observed hypermethylation of the cancer-specific gene tissue factor pathway inhibitor 2 (TFPI2). CONCLUSION: This finding suggests that if the DNA hypermethylation of pure pancreatic juice had been assayed before steroid therapy, it would have supported the diagnosis of pancreatic cancer, and steroid therapy could have been avoided.

A case of autoimmune pancreatitis associated with retroperitoneal fibrosis.

CONTEXT: Autoimmune pancreatitis is characterized by diffuse enlargement of the pancreas, diffuse irregular narrowing of the main pancreatic duct, severe lymphoplasmacytic infiltration and fibrosis of the pancreas. Retroperitoneal fibrosis may occasionally be associated with autoimmune pancreatitis. CASE REPORT: We report a 77-year-old man with autoimmune pancreatitis associated with retroperitoneal fibrosis. Abdominal ultrasonography and computed tomography demonstrated diffuse enlargement of the pancreas and a capsule-like rim. Furthermore, a retroperitoneal mass was recognized anterior to the abdominal aorta. Antinuclear antibody, IgG and IgG4 values were elevated. Therefore, this patient was diagnosed as having autoimmune pancreatitis associated with retroperitoneal fibrosis. We performed steroid therapy using prednisolone. After 4 weeks, both IgG and IgG4 values decreased and both the swelling of the pancreas and also the retroperitoneal mass were obviously diminished. CONCLUSION: This is a rare case of autoimmune pancreatitis associated with retroperitoneal fibrosis.