Gangliocytic Paraganglioma with Carcinoma of the Ampulla of Vater.

The patient was a "73" -year-old woman who visited our hospital with the chief complaint of weight loss. Upper gastrointestinal endoscopy revealed an enlarged ampulla of Vater, and a biopsy led to a diagnosis of Group "4" gastric carcinoma; suspicious of adenocarcinoma. There were no findings suggesting invasion into the muscle layer of duodenum, despite tumor mass formation being observed in the sphincter of Oddi. We performed endoscopic papillectomy for both diagnostic and therapeutic purposes. Pathologically, a well-differentiated adenocarcinoma existed in the superficial layer of the mucous membrane of the papilla of Vater, and gangliocytic paraganglioma was present in the deep portion. The resected margins of both lesions were negative.

The role of Gαq/Gα11 signaling in intestinal epithelial cells.

Intestinal homeostasis and the coordinated actions of digestion, absorption and excretion are tightly regulated by a number of gastrointestinal hormones. Most of them exert their actions through G-protein-coupled receptors. Recently, we showed that the absence of Gαq/Gα11 signaling impaired the maturation of Paneth cells, induced their differentiation toward goblet cells, and affected the regeneration of the colonic mucosa in an experimental model of colitis. Although an immunohistochemical study showed that Gαq/Gα11 were highly expressed in enterocytes, it seemed that enterocytes were not affected in Int-Gq/G11 double knock-out intestine. Thus, we used an intestinal epithelial cell line to examine the role of signaling through Gαq/Gα11 in enterocytes and manipulated the expression level of Gαq and/or Gα11. The proliferation was inhibited in IEC-6 cells that overexpressed Gαq/Gα11 and enhanced in IEC-6 cells in which Gαq/Gα11 was downregulated. The expression of T-cell factor 1 was increased according to the overexpression of Gαq/Gα11. The expression of Notch1 intracellular cytoplasmic domain was decreased by the overexpression of Gαq/Gα11 and increased by the downregulation of Gαq/Gα11. The relative mRNA expression of Muc2, a goblet cell marker, was elevated in a Gαq/Gα11 knock-down experiment. Our findings suggest that Gαq/Gα11-mediated signaling inhibits proliferation and may support a physiological function, such as absorption or secretion, in terminally differentiated enterocytes.

Tacrolimus versus anti-tumor necrosis factor agents for steroid-refractory active ulcerative colitis based on the severity of endoscopic findings: a single-center, open-label cohort study.

BACKGROUND AND AIMS: At Saitama Medical Center, for remission induction in active ulcerative colitis (UC) patients with endoscopic evidence of severe disease, we tend to preferentially use tacrolimus (TAC) over anti-tumor necrosis factor (TNF)-α agents. We conducted this study to evaluate the validity of our therapeutic strategies. PATIENTS AND METHODS: This retrospective study was conducted in 52 steroid-refractory active UC patients with a Clinical Activity Index (CAI) score of ≥7 who were receiving remission induction therapy with TAC or anti-TNF-α agents. The patients were divided into a TAC treatment group (TAC group, n = 29) and an anti-TNF-α agent treatment group (anti-TNF group, n = 23). The CAI, Ulcerative Colitis Endoscopic Index of Severity (UCEIS) and incidence of events (relapse, hospitalization and surgery) were retrospectively analyzed. RESULTS: At treatment initiation, the CAI score was 12.6 in the TAC group and 11.5 in the anti-TNF group (P = 0.09), while the corresponding values of the UCEIS were 6.5 and 5.1, respectively (P = 0.0035). The clinical remission rate at 12 weeks was 55% (65% when only the subgroup that received rapid induction therapy was included in the analysis) in the TAC group and 57% in the anti-TNF group, with no significant difference. The cumulative event-free rates at 1, 6 and 12 months were 65.5%, 39.4%, and 39.4%, respectively, in the TAC group and 95.7%, 77.2% and 71.7%, respectively, in the anti-TNF group (P = 0.0037). CONCLUSION: Rapid induction therapy with TAC tended to be selected for active UC patients with endoscopic evidence of severe disease, and the present study supported the validity of this therapeutic approach. However, transition to the remission-maintenance phase was more favorable in the anti-TNF group.

[A case of primary splenic angiosarcoma with intraperitoneal hemorrhage treated by transcatheter arterial embolization].

Splenic tumors are very rare. In Japan only 42 cases of splenic angiosarcoma have been reported. We encountered a case of spontaneous rupture of a splenic angiosarcoma and liver metastasis. A 60-year-old woman who suddenly went into hemorrhagic shock presented at our hospital. Then acute spontaneously ruptured spleen and hepatic tumors were diagnosed by abdominal CT. After emergency TAE, the patient was hemodynamically stable, but died of liver failure 13 days after admission. The pathological diagnosis was primary splenic angiosarcoma with multiple organ metastasis on autopsy. Splenic angiosarcoma should be kept in mind in the differential diagnosis of splenomegaly or splenic tumor. TAE can be effective in primary hemostasis for angiosarcoma with intraperitoneal hemorrhage from multiple tumors.

[Endoscopic ultrasound-guided transmural cyst drainage (EUS-CD) was effective in a case of pancreatic pseudocyst with obstructive jaundice and duodenal stenosis].

A 40-year-old man was admitted to our hospital because of epigastralgia and vomiting. His condition was diagnosed as acute pancreatitis with a pancreatic pseudocyst, obstructive jaundice, and duodenal stenosis. Because he had fever, abdominal pain, and elevated levels of C-reactive protein (CRP), endoscopic ultrasound-guided transmural cyst drainage (EUS-CD) was performed with a nasocystic tube on the 6th day. After the cyst was reduced and the patient recovered from the obstructive jaundice and duodenal stenosis, the nasal drainage tube was replaced with a plastic stent. Because a short extent of stenosis in the main pancreatic duct in the pancreatic head was found by endoscopic retrograde cholangiopancreatography (ERCP), a 5Fr pancreatic stent was placed to prevent pancreatitis. No recurrence of pancreatitis and the cyst occurred after removal of both stents 5 days later.

Slow growing flat-type primary malignant melanoma of the esophagus treated with cap-assisted EMR.

We report a rare case of flat-type primary malignant melanoma of the esophagus treated with endoscopic mucosal resection (EMR). A 64-year-old woman was referred for examination of a small pigmented lesion located in the mid esophagus. On endoscopy, the lesion exhibited almost no change in size over the year. Cap-assisted EMR was performed en bloc. The histopathological findings showed atypical melanocyte proliferation in the basal layer, spindle cells, and epithelioid cell proliferation with nuclear enlargement and a few mitotic figures. Histopathological examination confirmed the diagnosis of primary malignant melanoma. Immunostaining for S-100 protein and HMB-45 antibody were positive, and the Ki-67 index was low. The patient was discharged without additional surgical resection and/or chemotherapy. The patient had no symptoms and no signs of recurrence 20 months after EMR. There has been no report on a slow growing esophageal melanoma. It is difficult to estimate the malignant behavior of this case.

Biliopancreatic fistula with portal vein thrombosis caused by a pancreatic pseudocyst.

We encountered a very rare case of biliopancreatic fistula with portal vein thrombosis caused by pancreatic pseudocyst. A 57-year-old man was referred to our hospital because of abdominal pain, obstructive jaundice, and portal vein thrombosis due to acute pancreatitis. Computed tomography showed a 7-cm-diameter pseudocyst around the superior mesenteric vein extending towards the pancreatic head, dilatation of the intrahepatic bile duct, and portal vein thrombosis. Endoscopic retrograde pancreatography revealed a main pancreatic duct with a pseudocyst communicating with the common bile duct. After pancreatic sphincterotomy, a 7-F tube stent was endoscopically placed into the pseudocyst. However, a 6-F nasobiliary tube could not be inserted into the bile duct because the fistula had a tight stenosis. Subsequently, the patient's abdominal pain improved, the pancreatic cyst disappeared, and the serum amylase level normalized. Two months after the endoscopic retrograde cholangiopancreatography, percutaneous transhepatic biliary drainage was required because the patient's jaundice became aggravated. Two weeks after the choledochojejunostomy, the patient left the hospital in good condition. A follow-up computed tomography showed cavernous transformation of the portal vein and no pancreatic pseudocyst. The patient remains asymptomatic for 2 years and 7 months after surgery. Biliary drainage may be necessary for biliopancreatic fistula with obstructive jaundice in addition to pancreatic cyst drainage. Biliopancreatic fistula can be treated by endoscopic procedure in some cases; however, surgical treatment should be required in cases that are impossible to insert a biliary stent because of hard stricture.

Hepatocellular carcinoma with direct invasion to the stomach.

A 71-year-old man was admitted to our hospital with abdominal pain. Hepatocellular carcinoma (HCC) had been diagnosed 2 years earlier and he had undergone 7 courses of intra-hepato-arterial chemotherapy (IHAC). We performed gastrointestinal fiberscopy and identified a massive protrusion on the lesser curvature. Abdominal contrast-enhanced computed tomography revealed multiple hepatic masses and an extrahepatic enlarged mass with invasion to the pancreas and stomach. A specimen for endoscopic biopsy revealed adenocarcinoma that stained positive for alpha-fetoprotein. Gastrointestinal bleeding resulting from direct invasion of HCC is unusual.