A case of malignant peritoneal mesothelioma with a Fitz-Hugh-Curtis syndrome-like imaging finding.

Malignant peritoneal mesothelioma (MPeM) is a rare disease with a poor prognosis that develops in the mesothelial cells of the peritoneum. We encountered a 48-year-old man with no prior asbestos exposure who visited our hospital with abdominal pain. Laboratory findings showed elevated C-reactive protein of 15.5 mg/dL. Contrast-enhanced computed tomography (CT) detected a Fitz-Hugh-Curtis syndrome-like contrast effect on the liver surface and thickening of the peritoneum. Blood culture, Mycobacterium tuberculosis-specific IFN-γ release assay, Chlamydia trachomatis and Neisseria gonorrhoeae DNA testing, and antinuclear antibody were all negative. CA125 was high at 124.8 U/mL. The laparoscopy for diagnostic purposes revealed adhesions between the liver surface and peritoneum in addition to numerous small and large white nodules on the peritoneum. Biopsy of the nodules confirmed the diagnosis of epithelial-type MPeM. Treatment was initiated with combined cisplatin and pemetrexed, and CT 6 months later showed a reduced contrast effect on the liver surface and improved peritoneal thickening. A Fitz-Hugh-Curtis syndrome-like contrast effect on the liver surface on contrast-enhanced CT may help identify MPeM.

Rapid identification of source of delayed hemobilia following endoscopic metallic stenting for malignant biliary obstruction.

Delayed hemobilia, a rare but potentially fatal complication of endoscopic metallic stenting for malignant biliary obstruction, requires prompt identification of the source of bleeding and subsequent embolization. However, hemobilia is characteristically intermittent, and computed tomography (CT) often fails to show pseudoaneurysms or extravasations. In particular, because the posterior superior pancreaticoduodenal artery (PSPDA) runs alongside the common bile duct for its whole length, it is readily obscured by metallic artifacts in that duct, such as stents, making identification of the source of bleeding by CT difficult. We have encountered three patients with delayed hemobilia from the PSPDA following endoscopic biliary stenting for malignant biliary obstruction in whom no extravasation or pseudoaneurysms were detected by contrast-enhanced CT during bleeding. However, when we identified that the PSPDA had a smaller diameter than in previous CTs in all three cases, we suspected that the PSPDA was the source of the bleeding. No extravasation or pseudoaneurysms were detected with celiac arteriography or superior mesenteric arteriography; however, extravasation and pseudoaneurysms were detected by direct PSPDA angiography. Hemostasis was achieved through embolization. Detecting a large decrease in the diameter of the PSPDA on contrast-enhanced CT during biliary bleeding may help to identify the source of that bleeding.

[A case of "colitis-like intestinal Behçet's disease" accompanied by ulcerative colitis after the development of myelodysplastic syndrome].

A 71-year-old man developed ulcerative colitis (UC) at 48 years of age. As a steroid-dependent case with poor UC control, the patient was treated with azathioprine, which resulted in clinical remission. However, a blood test revealed pancytopenia. Bone marrow examination confirmed the diagnosis of myelodysplastic syndrome (MDS). During the patient's clinical course, multiple round ulcers appeared in the terminal ileum. We suspected concomitant "colitis-like intestinal Behçet's disease" (BD). Treatment with adalimumab resolved the ulcers. To the best of our knowledge, this is a rare case of intestinal BD accompanying UC after MDS.

Massive ascites caused by intra-pancreatic arterioportal fistula: a rare complication of chronic pancreatitis.

An 86-year-old man with a long-term habit of ethanol consumption was admitted due to massive transudate ascites and leg edema. Abdominal computed tomography revealed a dilated main pancreatic duct and atrophied pancreatic parenchyma, leading to the diagnosis of chronic pancreatitis. Moreover, the portal vein was enhanced in the early arterial phase, which indicated the presence of an arterioportal fistula. The fistula was located between the posterior superior pancreaticoduodenal artery and the portal vein near a pancreatic retention cyst. Transarterial coil embolization dramatically improved the ascites. Arterioportal fistula and ensuing ascites should be recognized as a complication of chronic pancreatitis.

A case of simultaneous esophageal squamous cell carcinoma and Barrett's adenocarcinoma.

A 77-year-old male with a long history of alcohol consumption and smoking was admitted for hoarseness and dysphagia. Computed tomography revealed thickening of the middle intrathoracic esophageal wall and multiple mediastinal lymph node swellings. Esophagogastroduodenoscopic examination disclosed an advanced-stage squamous cell carcinoma lesion in the middle intrathoracic esophagus with synchronous early stage Barrett's adenocarcinoma. The patient underwent endoscopic submucosal dissection for the adenocarcinoma followed by chemoradiation therapy for the squamous cell carcinoma. In spite of their common risk factors, the simultaneous manifestation of esophageal squamous cell carcinoma and Barrett's adenocarcinoma is extremely rare and requires further study.

Case of Barrett's adenocarcinoma with marked endoscopic morphological changes in Barrett's esophagus over a long follow-up period of 15 years.

The natural history of Barrett's esophagus (BE) is unclear. We herein describe a case of Barrett's adenocarcinoma (BAC) in which we could closely observe marked morphological changes in BE over a long follow-up period of 15 years. A man in his seventies received routine esophagogastroduodenoscopy (EGD) and was diagnosed as having reflux esophagitis and short-segment BE. The BE gradually became elongated, and BAC was detected 9 years following the initial EGD examination with continued administration of a proton pump inhibitor. We witnessed that BE elongated sporadically over time and mucosal breaks of reflux esophagitis were detectable several years before elongation. The patient underwent endoscopic submucosal dissection for BAC and has been monitored by EGD every year thereafter. These remarkable morphological changes may be representative of the natural history of BE and aid in deciding long-term disease management.

Helicobacter pylori-negative Differentiated Adenocarcinoma of the Stomach.

A 58-year-old Japanese man was diagnosed with differentiated adenocarcinoma of the stomach. Histological findings of the resected specimen revealed well- to moderately-differentiated tubular adenocarcinoma (tub1, tub2), 13 mm in diameter, which invaded into the submucosa (SM1, 300 µm) and lymphovascular lumen (ly1). Serum antibody against Helicobacter pylori (Hp) and the (13)C-urea breath test were negative, and there were no atrophic changes in the tumor-adjacent mucosa. The immunohistochemical analysis showed that gastric mucin (MUC5AC) was strongly positive and intestinal mucin (MUC2) was weakly and partially positive. According to these results, the final diagnosis of Hp-negative well-differentiated early gastric cancer was made.

[A case of metachronous invasive ductal carcinoma concomitant with intraductal papillary-mucinous neoplasm (IPMN) of the pancreas, which could not be detected in contrast-enhanced CT scan performed 3 months ago].

A 61-year-old man had been followed up in another hospital under diagnosis of branch duct type IPMN for 4 years. Contrast-enhanced CT scan for regular check performed 3 months ago revealed no increase of IPMN and no pancreatic tumor. However, he complained of back pain after that, MRI was performed. It revealed a solid tumor in size of 25mm diameter at the head of pancreas. The tumor was apparent from IPMN in several imaging modalities. Pancreatoduodenectomy was performed under diagnosis of invasive ductal carcinoma concomitant with IPMN. Post-operative pathological findings revealed IPMN was adenoma with mild atypia, and solid tumor was diagnosed invasive ductal carcinoma with solitary minute liver metastasis.

Efficacy of preoperative endoscopic nasobiliary drainage for hilar cholangiocarcinoma.

BACKGROUND/PURPOSE: Although percutaneous transhepatic biliary drainage has previously been recommended as a primary preoperative step, endoscopic nasobiliary drainage (ENBD) is prevalent as an alternative procedure. Few reports assess the efficacy and safety of ENBD in a substantial patient cohort. METHODS: Of 116 patients with hilar cholangiocarcinoma who underwent surgery, 62 (43 men and 19 women, median age 69 years) underwent preoperative ENBD. After classification of lesions according to Bismuth-Corlette (B-C) criteria, we evaluated efficacy and safety with respect to B-C type. RESULTS: Patients were classified as B-C types I (n = 5), II (n = 21), IIIa (n = 23), IIIb (n = 5), and IV (n = 8). Preoperative single ENBD was effective in 46/62 patients (74%) including 5/5 (100%) B-C type I, 20/21 (94%) type II, 16/23 (70%) type IIIa, 4/5 (80%) type IIIb, and 1/8 (13%) type IV. Sixteen cases (26%) required additional drainages with ENBD or endoscopic biliary stenting (EBS) in 8/16 (50%), and with PTBD in 8/16 (50%). Mild acute pancreatitis (n = 1, 2%), segmental cholangitis (n = 2, 3%), and acute cholangitis with catheter obstruction (n = 7, 11%) occurred with ENBD. CONCLUSIONS: Preoperative single ENBD in the future remnant lobe is effective treatment for B-C type I-III hilar cholangiocarcinoma. Preoperative ENBD was rarely complicated with segmental cholangitis.

[Survey of gemcitabine treatment for advanced pancreatic cancer in 20 hospitals of Nagano Prefecture].

To establish an effective therapy for pancreatic cancer, we made a retrospective survey of gemcitabine treatment performed at 20 hospitals in Nagano Prefecture. We analyzed data of 106 patients (64 men and 42 women, median age 66 years (33-83 years old)), half of whom had stage IV disease. Gemcitabine was administered for 3 consecutive weeks with one week rest in 57 patients, biweekly in 30 patients, initially for 3 weeks with 1 week rest and switched to biweekly schedule to 15 patients, and with another regimen to 4 patients. Analysis of the results of gemcitabine treatment between the 3 weeks with 1-week-rest regimen and the biweekly regimen revealed no differences between regimens in growth inhibition and symptom alleviation. However, we found less occurrence of blood toxicity in the biweekly regimen (40%) than in the 3 weeks with 1 week-rest regimen (59%). Median survival time for the biweekly regime was 9.7 months, only slightly longer than that of the 3 weeks with 1-week-rest regimen (8.5 months). The present study showed that a biweekly regimen for gemcitabine administration may be equivalent to the standard regime of 3 weeks with 1-week-rest regimen. Moreover, the biweekly regimen has advantages over the 3 weeks with 1-week rest regimen both economically and in terms of convenience for outpatient treatment. Therefore, the present results should be confirmed in future prospective studies, with the hope of developing a new standard treatment regimen for pancreatic cancer.

Corticosteroid-responsive pancreatic cyst found in autoimmune pancreatitis.

There have been only a few reports of autoimmune pancreatitis complicated with pancreatic cyst and such cysts are rare, probably due to the absence of severe tissue necrosis and/or lack of stasis of the pancreatic juice in this condition. However, during a follow-up of 48 patients with this disease, we found 3 patients with pancreatic cysts, and this enabled us to evaluate their clinicopathological findings. Between September 1994 and July 2003, we treated and followed 48 patients with autoimmune pancreatitis, and found 3 patients with pancreatic cyst formation that was responsive to corticosteroid therapy. All of the patients with cysts had high serum IgG4 concentrations. After corticosteroid therapy, rapid resolution of the pancreatic cysts was observed. Immunostaining with goat polyclonal antibody for each IgG subclass showed severe infiltration of IgG4-positive plasma cells in the cyst wall in one patient. The high serum IgG4 concentration and favorable response to corticosteroid therapy suggests that a highly active state of the inflammatory process is closely associated with cyst formation, and that a corticosteroid-responsive pancreatic cyst is a characteristic feature of autoimmune pancreatitis.

Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis?

BACKGROUND: Autoimmune pancreatitis has been designated as sclerosing pancreatocholangitis, because this disease shows a high prevalence of bile-duct lesions. We present herein the clinical characteristics of unusual cases that show dominant bile-duct lesions and mimicking infiltrating hilar cholangiocarcinomas. METHODS: Clinical and pathologic findings of 3 patients with immunoglobulin (Ig) G4 related sclerosing cholangitis who had no apparent pancreatic lesions comparable with autoimmune pancreatitis were analyzed. OBSERVATIONS: All patients were middle-aged or elderly individuals with slightly elevated serum IgG4 concentrations and showed long-segment narrowing of the bile-duct system, mimicking infiltrating hilar cholangiocarcinoma without significant pancreatic change. The first patient was treated with a corticosteroid, resulting in amelioration of the narrowing of the bile duct. The second patient underwent surgery based on a diagnosis of cholangiocarcinoma. In the third patient, the bile-duct stricture reversed spontaneously 1 month after the drainage procedure. Pathologic findings of the bile ducts for all patients disclosed significant lymphoplasmacytic infiltration, including abundant IgG4-bearing plasma cells. CONCLUSIONS: The use of IgG4 immunostaining in biopsy specimens of the bile duct may identify the presence of corticosteroid-responsive lymphoplasmacytic sclerosing cholangitis.

Possible relationship between Helicobacter pylori infection and cap polyposis of the colon.

BACKGROUND: Cap polyposis is a rarely encountered disease characterized by multiple distinctive inflammatory colonic polyps located from the rectum to the distal colon. The etiology of this disease is still unknown, and no specific treatment has been established. AIM: We report three cases of cap polyposis that were cured following eradication therapy for Helicobacter pylori infection. METHODS AND RESULTS: Three women were referred to Shinshu University Hospital because of mucoid and/or bloody diarrhea. Laboratory data showed hypoproteinemia in all cases; markers of inflammation such as C-reactive protein were negative. Colonoscopy revealed multiple sessile polyps with mucus adherent on the apices of the mucosal folds in the rectum and/or the sigmoid colon. The intervening mucosa was normal. Microscopic examinations of biopsy specimens taken from sessile polyps revealed inflamed mucosa with elongated tortuous crypts attenuated towards the mucosal surface. A granulation tissue 'cap' was observed on the surface of the mucosa. Various treatments were unsuccessful, including administration of metronidazole or prednisolone, avoidance of straining at defecation, and surgical or endoscopic resection. All were diagnosed with H. pylori infection in the stomach. Helicobacter pylori was not detected in the biopsy specimens from the colonic inflammatory polyps by immunohistochemical study using polyclonal anti-H. pylori antibody. After successful eradication therapy the clinical symptoms improved. Disappearance of cap polyposis was confirmed by colonoscopy in all three cases. CONCLUSION: We speculate that H. pylori infection might play a role in the pathogenesis of cap polyposis.

[Clinical problems in gemcitabine treatment for unresectable pancreatic cancer in the elderly--a multicentric retrospective study of 53 cases].

In Gemcitabine treatment, elderly patients with unresectable pancreatic cancer are more likely to suffer from haematological and non-haematological adverse effects than non-elderly patients. Forty percent of the elderly patients were dropped from the initial protocol due to the adverse effects, mainly because of non-haematological events or symptoms. To avoid adverse effects, the administration schedule for Gemcitabine tended to be less often and at a lower dose for elderly patients among members of the Nagano Pancreatic Cancer Study Group. However, the fact that some cases showed a limited effect from this administration schedule albeit without adverse effect, might suggest that the frequency of ordinary administration schedule should be maintained, although the Gemcitabine dose could be decreased in unresectable pancreatic cancer patients in poor condition.

Extracorporeal shock wave lithotripsy of pancreatic duct stones and patient factors related to stone disintegration.

BACKGROUND: Stones in the main pancreatic duct (MPD) are difficult to remove by endoscopic devices alone in some patients who have chronic pancreatitis. We treated these patients with extracorporeal shock wave lithotripsy (ESWL) and analyzed the patient factors related to disintegration. METHODS: Twenty-four patients were treated with ESWL alone or with combined endoscopic-ESWL to disintegrate or remove MPD stones. RESULTS: Ten patients were treated by ESWL alone and 14 by combined endoscopic-ESWL. A total of 19 patients (79%) were effectively treated by either method. The mean MPD diameter decreased significantly after ESWL. In most of the patients who had chronic abdominal symptoms, these symptoms were relieved at discharge. Severe side effects of complications did not occur during ESWL therapy. Acute abdominal symptoms and a significant increase in the white blood cell count, total bilirubin, and aspartate aminotransferase were observed only immediately after ESWL. Although there were no significant differences, we observed that the patients with a higher stone disintegration success rate showed the following factors: (1) female, (2) non-alcoholic pancreatitis, (3) younger age, (4) shorter duration of symptoms, (5) smaller stones, and (6) a lower Hounsfield unit value of stones. Although about half of the patients had recurring abdominal symptoms and stones during a follow-up period of 12 months, the stones which caused relapse in short-term intervals were disintegrated easily by ESWL. CONCLUSIONS: We may consider the application of ESWL therapies for patients who show the factors associated with easily disintegratable stone conditions. These therapies are highly effective and relatively safe procedures for pancreatic duct stones in such patients.

HLA DRB10405-DQB10401 haplotype is associated with autoimmune pancreatitis in the Japanese population.

BACKGROUND & AIMS: Autoimmune pancreatitis is a distinctive disease entity characterized by high serum immunoglobulin G4 concentrations. Because of the close association between some autoimmune diseases and particular alleles of major histocompatibility complex genes, we investigated the association between HLA alleles and autoimmune pancreatitis. METHODS: HLA-A, -B, -C, -DR, and -DQ gene typing and HLA-DRB1, -DQB1, and -DPB1 allele typing were performed by the polymerase chain reaction sequence-specific primers method and the restriction fragment length polymorphism method, respectively, in 40 patients with autoimmune pancreatitis, 43 patients with chronic calcifying pancreatitis, and 201 healthy subjects. RESULTS: In patients with autoimmune pancreatitis compared with healthy subjects, we found a significant increase in DR4 (73% vs. 44%, corrected P = 0.01) and DRB1*0405 (58% vs. 21%, corrected P = 0.000026) and DQ4 (58% vs. 26%, corrected P = 0.001) and DQB1*0401 (58% vs. 21%, corrected P = 0.000017). The DRB1*0405-DQB1*0401 haplotype in autoimmune pancreatitis showed no significant association with any HLA class I antigens, in contrast to the B54-DRB1*0405-DQB1*0401 haplotype reported in autoimmune hepatitis. The frequencies of DRB1*0405 and DQB1*0401 were significantly high in patients with autoimmune pancreatitis compared with chronic calcifying pancreatitis. CONCLUSIONS: It is probable that DRB1*0405-DQB1*0401 haplotype is associated with autoimmune pancreatitis in the Japanese population.