Multiple gastrointestinal stromal tumors in neurofibromatosis type 1: report of a case.

This report presents a case of multiple gastrointestinal stromal tumors (GIST) with neurofibromatosis type 1 (NF1). A 68-year-old woman was admitted to the hospital because of a tumor close to the head of the pancreas. Imaging studies revealed submucosal tumors of the duodenum. The retroperitoneal tumor was diagnosed before surgery. Besides the main tumor in the duodenum, multiple small submucosal tumors were found in the duodenum and upper part of the jejunum during the operation. All of these tumors were resected. The histological diagnosis of all these tumors was GISTs. These tumors were immunohistochemically positive for KIT, but they demonstrated no mutation in c-kit exons 9, 11, 13, and 17, and platelet-derived growth factor receptor alpha exons 12 and 18. No recurrence occurred for a year after surgery.

Spleen and gastrosplenic ligament preserving distal pancreatectomy under a minimum incision approach assisted by laparoscopy.

BACKGROUND: As a modification of hand-assisted laparoscopic pancreatectomy, we devised a method of spleen and gastrosplenic ligament preserving distal pancreatectomy, in which pancreatic resection is performed under direct vision extracorporeally. METHODS: The distal pancreas and spleen are pulled out of the peritoneal cavity through the minilaparotomy at the epigastrium following hand-assisted laparoscopic dissection of the distal pancreas. Spleen-preserving pancreatectomy is performed safely under direct vision. The gastrosplenic ligament is also preserved to prevent splenic volvulus after the operation. The transected main pancreatic duct is doubly ligated, and the transected pancreatic stump is sewn manually. The preserved spleen and splenic vessels are placed back in the peritoneal cavity after resection. RESULTS: In the current study (n = 3), overall morbidity rate, including splenic volvulus and pancreatic fistula, was 0%. CONCLUSION: Preservation of the gastrosplenic ligament and extracorporeal preparation of the transected pancreatic stump under direct vision are useful measures in spleen-preserving distal pancreatectomy under a minimum incision approach assisted by laparoscopy.

Combined chemotherapy of irinotecan and low-dose cisplatin (I/low-P) against metastatic biliary tract cancer.

There is no established or effective standard therapy for metastatic biliary tract cancer, resulting in poor prognosis. Recently, we performed combination chemotherapy of irinotecan and low-dose cisplatin (I/low-P) for three consecutive patients with metastatic biliary tract cancer. The regimen of I/low-P therapy consisted of irinotecan (60 mg/m(2)) and low-dose cisplatin (6 mg/m(2)), administered by intravenous infusion weekly or biweekly. Of the three patients, two showed a partial response, with durations of more than 20 months, and 2 months, respectively, while the third patient had stable disease for 3 months. One patient, who had jaundice, had grade 3 thrombocytopenia, but the other patients did not have any severe toxicities. Survival times were more than 20 months, 10 months, and 13 months, respectively. These outcomes suggest that I/low-P therapy is safe and may be worth trying as a first-line chemotherapy for patients with metastatic biliary tract cancer.

Applicability of disseminated intravascular coagulation parameters in the assessment of the severity of acute pancreatitis.

OBJECTIVES: To evaluate the clinical applicability of the determination of disseminated intravascular coagulation (DIC) parameters in acute pancreatitis. METHODS: The subjects for this study were 139 consecutive patients with acute pancreatitis. DIC parameters were assessed at the initial observation of these patients. RESULTS: The levels of the DIC parameters at admission were significantly associated with the severity and the prognosis of acute pancreatitis. Antithrombin III (AT-III), fibrin/fibrinogen degradation products-E, platelet count, D-dimer, and thrombin-AT-III complex at admission showed better area under the receiver operating characteristics curve values compared with C-reactive protein. An AT-III value of 69% at admission was the best cut-off value to predict fatal outcome (sensitivity, 81%; specificity, 86%). CONCLUSIONS: The aggravated coagulation parameters predict a fatal outcome in patients with acute pancreatitis. AT-III level (<69%) was the most accurate marker for poor outcome of acute pancreatitis at admission.

Proinflammatory role of trypsin and protease-activated receptor-2 in a rat model of acute pancreatitis.

OBJECTIVES: The pathophysiology of acute pancreatitis is strongly associated with autoactivation of trypsin. The biologic activity of trypsin on cells is attributed to the activation of protease-activated receptor-2 (PAR-2). We hypothesize that trypsin may activate acinar cells or inflammatory cells through PAR-2 signals in acute pancreatitis. METHODS: We immunochemically analyzed the expression of PAR-2 in the rat acinar cell line, ARIP, and the rat pancreas, using anti-rat PAR-2 cleavage site (PCS) and anti-rat PAR-2 N-terminal fragment (PNF) antibodies. Plasma levels of PNF were determined. Furthermore, the effects of the anti-rat PCS antibody and nafamostat mesylate, a potent trypsin inhibitor, on PAR-2 activation during acute pancreatitis were also analyzed. RESULTS: ARIP cells expressed PAR-2, which was activated by exogenous trypsin activity. We also showed that PAR-2 is strongly expressed in pancreatic acinar and duct cells and that it is activated in rat cerulein-induced acute pancreatitis. The anti-rat PCS antibody and nafamostat mesylate reduced interleukin-6 and interferon gamma production and alleviated distant organ injury. CONCLUSIONS: These results suggest that trypsin and its specific receptor, PAR-2, play an important role in cytokine production and the resultant development of distant organ injury during rat acute pancreatitis.