Naoto Urushihara

Frantz's tumor: A papillary and cystic tumor of the pancreas in girls

Four girls with Frantzs tumor, a papillary and cystic tumor of the pancreas, are studied and discussed in comparison with 112 cases in the literature, including 58 Japanese cases. The neoplasms occur predominantly in girls and young women. Up to recently, the tumors have possibly been misclassified as nonfunctioning islet cell tumor or carcinoma, acinar cell carcinoma, papillary cystadenocarcinoma, or pancreatoblastoma according to microscopic findings and frequently have been managed with aggressive surgery such as pancreatoduodenectomy. The tumors are well encapsulated and the cut surfaces show characteristically solid and hemorrhagic-necrotic patterns. Ultrasonography and CT scan are the most useful tools for the diagnosis. The neoplasms usually behave like a very low-grade malignancy, so that complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. Immunochemical and electron microscopic studies can differentiate Frantzs tumor from other neoplasms and also suggest that the tumors originate from primordial cells or multipotential stem cells capable of differentiating into both exocrine and endocrine lines.

Elevation of serum interleukin-18 levels and activation of kupffer cells in biliary atresia

BACKGROUND/PURPOSE Interleukin-18 (IL-18)/interferon-gamma-inducing factor (IGIF) is a novel proinflammatory cytokine that can induce interferon gamma (IFN-gamma). In addition, IL-18 enhances intracellular adhesion molecule-1 (ICAM-1) expression as well as Fas ligand (FasL) expression, and induces apoptosis in hepatic injury. The aim of this study was to clarify the potential role of IL-18 in the pathogenesis of the progressive inflammation and fibrosis in biliary atresia (BA). METHODS Six children with BA before hepatic portoenterostomy (HPE), 13 with BA including 7 without jaundice and 6 with persistent jaundice after HPE, and 16 healthy controls were examined. Blood samples were obtained preoperatively from 6 patients, after HPE from 13, and after liver transplantation from 4. The IL-18 level was determined by an enzyme-linked immunosorbent assay (ELISA). Immunohistochemically, liver specimens from BA patients were studied using a monoclonal antibody to macrophage-associated antigen (CD68). RESULTS IL-18 levels were elevated in the patients before HPE compared with those of the controls (349+/-54 pg/mL v. 138+/-13 pg/mL, P<.0001). After HPE, extremely high concentrations of IL-18 were observed in patients with persistent jaundice (532+/-95 pg/mL, P<.0001), and the IL-18 levels were significantly high even in the patients without jaundice (249+/-29 pg/mL, P<0.005). The high IL-18 level lasted for a long time even in the patients without jaundice after HPE. In contrast, the IL-18 levels immediately decreased after liver transplantation. Immunohistochemically, the number of CD68-positive Kupffer cells was significantly higher, and the size was larger in the livers of the patients than in the controls. The proliferation of CD68-positive cells was much more conspicuous in the liver specimens obtained during liver transplantation than in those at the time of HPE. CONCLUSIONS Our findings showed elevation of serum IL-18 levels and activation of Kupffer cells in BA. IL-18 released from activated Kupffer cells might play an important role in the pathophysiology of the progressive inflammation and fibrosis in BA. Furthermore, IL-18 level may be related to the prognosis in patients with BA.

Choledochal cyst, pancreatobiliary malunion, and cancer

A review of the clinical features of biliary cancer with choledochal cyst and pancreatobiliary malunion is presented, together with a recent case report. Biliary cancer develops in about 25% of patients with choledochal cyst and pancreatobiliary malunion, and usually occurs in younger patients (usually those in their 40s) than does biliary carcinoma in the general population. The risk of malignancy in the retained cyst with internal drainage is higher than that in the primary cyst. Early excision of the retained cyst should be performed as quickly as possible, even if the patient is symptomless. Some bile acid fractions and refluxed pancreatic enzymes in bile are possibly responsible for carcinogenesis. Carcinoma generally develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic bile duct. In cystic dilatation, cancer usually occurs in the common bile duct, while in diffuse or non-dilated type, it develops in the gallbladder. Multicentric carcinomas develop in the bile duct either synchronously or metachronously. The prognosis of biliary cancer is usually dismal. However, aggressive procedures are now gaining better results than conventional approaches. The procedure of choice for choledochal cyst or malunion is to prevent the development of cancer by performing an early excision. Removal of the entire extrahepatic bile duct is necessary, even in patients with malunion and no biliary dilatation. Carcinoma rarely arises in the intrahepatic bile duct after excisional surgery, probably due to the long-standing stricture of the bile duct. Capacious anastomosis and/or ductoplasty is essential. Carcinoma may also develop in the remnant bile duct. Excision of the distal duct extending into the pancreas is also necessary.

Involvement of interleukin-18 (IL-18) in mixed lymphocyte reactions (MLR).

The in vitro mixed lymphocyte reaction (MLR) is a useful model to study alloresponsiveness to histocompatibility antigens. Secretion of different cytokine proteins in the supernatant of allo-MLR cultures has been reported in a few studies. We studied the levels of the cytokines interferon gamma (IFN-gamma) and interleukin-6 (IL-6), IL-10, IL-12, and IL-18 in the supernatant in allo-MLR by ELISA assay. Supernatant levels of IFN-y, IL-6, IL-10, and IL-18 were detected at 12 h after MLR and markedly increased thereafter. In contrast, secretion of IL-12 was detected after 48-72 h. These results suggested that IFN-gamma production depended on IL-18 in the early phase of MLR and depended on both IL-18 and IL-12 in the late phase. An antibody (Ab) neutralizing test was also performed. The levels of IFN-gamma were significantly downregulated after the addition of anti-IL-18 Ab, anti-IL-12 Ab, or anti-IFN-y Ab, and the levels of IL-12 were significantly downregulated after the addition of anti-IL-12 Ab and anti-IL-18 Ab. Treatment with these Ab did not suppress IL-6 production at all. The two-way MLR showed the same tendency as the one-way MLR. These results suggest the importance of IL-18 and IL-12 in allogeneic cell interactions and also suggest the usefullness of these Ab as regulators of alloresponsiveness.

Peritoneal Lavage Versus Drainage for Perforated Appendicitis in Children

A total of 231 children with acute appendicitis were treated at our hospitals during the 10 years between 1984 and 1993, 53 of whom had a perforated appendix. These 53 patients were randomly assigned to two groups at the time of surgery according to the different procedures performed. Thus, 29 children were managed by appendectomy followed by peritoneal lavage using a large amount of saline, and intravenous antibiotic therapy consisting of aminoglycoside and cephem (lavage group), while the other 24 children were treated by appendectomy with silicon tube drainage and the same systemic antibiotic therapy (drainage group). The mean length of hospitalization, and the mean durations of fever and the need for fasting after laparotomy in the lavage group were significantly less than those in the drainage group: 10.1 versus 18.8 days, 2.8 versus 7.7 days, and 1.8 versus 3.5 days, respectively. The operation wounds healed well in the lavage group due to the fact that there was no drain. Wound infections occurred in two children from the lavage group and six from the drainage group. Intra-abdominal abscesses occurred in two children from the drainage group. Accordingly, peritoneal lavage appears to be superior to intraperitoneal tube drainage for the management of perforated appendicitis in children.

Rapidly enlarging esophageal duplication cyst

Abstract: We report a rare esophageal duplication cyst, in a 12-year-old girl. The cyst had enlarged rapidly within 2 years. In December 1997, on admission, computed tomography and magnetic resonance imaging demonstrated a cystic mass in the pleural cavity. We resected the cyst and the adjacent lung. Histopathological examination revealed an esophageal duplication cyst. Her presenting symptoms of fever and cough may have been related to infection of the cyst.

Successfully resected hepatoblastoma in a young adult with chronic hepatitis B : report of a case

Hepatoblastoma usually occurs in children, but a few cases have also been reported in adults. We report the unusual case of hepatoblastoma in an 18-year-old adult with chronic hepatitis B. He visited a local hospital with right upper abdominal pain. Abdominal ultrasound showed a large mass in the right lobe of his liver. He was referred to our hospital and admitted for further examination. At admission, liver function tests gave slightly elevated results (aspartate aminotransferase (AST) 103 IU/l, alanine aminotransferase (ALT) 63 IU/l). A test for hepatitis virus revealed that he was a hepatitis B surface antigen (HBsAg) carrier and had experienced seroconversion. His alpha-fetoprotein (AFP) was elevated to 1 548 000 IU/ml. Abdominal ultrasound showed a 109 x 96 x 80-mm mass with mosaic pattern in the right lobe of the liver and right portal vein thrombus. Abdominal computed tomography (CT) demonstrated a large low-density mass occupying the right lobe, with some high-density parts that showed calcification. From these results, we diagnosed hepatoblastoma in a young adult. A right lobectomy was performed. Pathological examination showed a highly differentiated hepatoblastoma. Adjuvant chemotherapy was performed with cisplatin and pirarubicin. The patient has been well and free of recurrence for 12 months, and his AFP level remains almost normal.

Alveolar rhabdomyosarcoma of the lung in a child

The case of a 22-month-old boy with alveolar rhabdomyosarcoma of the lung is presented. Brain metastasis and recurrence of the right pulmonary hilum and parietal pleura developed 6, 11, and 24 months (respectively) after tumor resection. Chemotherapy and radiotherapy were effective. Neuron-specific enolase was very helpful in detecting metastasis and disease recurrence. Primary pulmonary rhabdomyosarcoma can be divided into two groups: tumor in the normal lung, and tumor in cystic lesions of the lung.

Functional Expression of Fas and Fas Ligand on Human Colonic Intraepithelial T Lymphocytes

The expression of Fas, a cell surface receptor directly responsible for triggering cell death by apoptosis, and its ligand (FasL) was investigated on both human colonic intraepithelial T lymphocytes (IELs) and peripheral blood mononuclear lymphocytes (PBMLs). FACS analysis indicated that IELs have increased expression of Fas compared with PBMLs, together with the progress activation marker, CD45RO. A discrete fraction of freshly isolated IELs also constitutively expressed FasL, perhaps as a result of recent in vivo activation. Using monoclonal antibody APO2.7, which detects mitochondrial 7A6 antigen specifically expressed by cells undergoing apoptosis, we further investigated the apoptosis-inducing effect of anti-Fas monoclonal antibody (CH11) on both IELs and PBMLs. FACS analysis revealed that CH11 increased the percentage of apoptotic cells, in IELs but not in PBMLs. Culture with anti-FasL monoclonal antibody (4H9) significantly recovered cell viability in IELs, but not in PBMLs. These results indicate that IELs constitutively express both Fas and FasL and that Fas crosslinking generates signals resulting in apoptosis, outlining a potential mechanism involved in intestinal tolerance.

Congenital Right Diaphragmatic Hernias Through Posterolateral and Anterolateral Defects Associated With Extralobar Pulmonary Sequestration: A Case Report

Multiple diaphragmatic hernias in the unilateral diaphragm are extremely rare. The authors report a neonate with diaphragmatic hernias through two defects in the right diaphragm: a posterolateral defect without a hernia sac and an anterolateral defect with one. After excision of the anterolateral hernia sac, each defect was closed. Histology studies showed extralobar pulmonary sequestration in the removed hernia sac. The presence of sequestrated pulmonary tissue indicates the possibility of interference with the closure of the pleuroperitoneal canal and muscularization in the diaphragm, which may result in multiple defects.