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Lymphangiomas are rare, benign tumors of the lymphatic system, usually present in children aged 5 years and younger. Because they are asymptomatic until the mass enlarges to cause symptoms, most lymphangiomas are diagnosed at adulthood incidentally. We experienced a case of a 60-year-old man diagnosed with a cystic lymphangioma of the gallbladder, which was successfully resected without any complication. Magnetic resonance imaging and magnetic resonance cholangiopancreatography were very helpful for the diagnosis of the cystic lesion around the gallbladder as were ultrasonography and computed tomography scan. These showed a multi-lobulated cystic mass with intact cystic duct and bile duct in the gallbladder fossa. The patient underwent an open cholecystectomy and the histological findings were consistent with a cystic lymphangioma of the gallbladder. We here report the case of cystic lymphangioma of the gallbladder with a review of the literature.

This case series presents the use of acellular dermal matrix (ADM) as a barrier membrane in reconstructing non-spacemaking buccal dehiscences associated with simultaneous implant placement in locally deficient ridges. Five sites in four healthy nonsmoking patients were treated with a combination of the mucogingival pouch flap technique, sandwiched layering of mineralized human cancellous and cortical bone grafts, and ADM as a barrier membrane. Three sites encountered 2 to 4 mm of membrane exposure after 2 weeks of healing time. However, all sites were completely covered at 3 months. None of the cases exhibited implant exposure throughout the entire healing period. At the 6-month re-entry surgery, ADM-assisted guided bone regeneration achieved a mean of 86.5% height gain and critical bone thickness of 1.8 mm or greater, with clinical bone density equivalent to that of the native bone.

Colon cancer is the most serious intestinal complication in patients with Crohn's disease. Although an association between Crohn's disease and colon cancer has not been clearly defined, a number of studies in western countries reported an increased rate of colon cancer in patients with Crohn's disease. In Korea, Crohn's disease is rare when compared to western countries, and only a single case of colon cancer associated with Crohn's disease has been reported. We recently experienced a 66-year-old women with colon cancer associated with Crohn's disease.

Adenosquamous carcinoma of the papilla of Vater is a rare tumor and only a few cases have been reported so far. Here, we report a case of adenosquamous carcinoma in a 76-year-old male who presented with jaundice and right upper quadrant abdominal pain. Ultrasonography and enhanced abdominal CT scans showed dilated common bile duct (CBD) and intrahepatic bile duct (IHD) with a suspicious obstructing mass in distal CBD. On endoscopy, obstructing and ulcerated mass was noted on the papilla of Vater. Histopathological inspection of the biopsied specimens from mass showed adenosquamous cell carcinoma of the papilla of Vater. Since the patient refused operation, we inserted a self-expandable metallic stent in distal CBD. This is the first case report on adenosquamous carcinoma of the papilla of Vater in Korea.

Background/aims: Nonalcoholic steatohepatitis can develop from nonalcoholic fatty liver and progress to severe liver disease such as cirrhosis. The mechanism determining the progression from fatty liver to steatohepatitis is unknown. Iron is suspected to enhance hepatic damage associated with nonalcoholic fatty liver disease (NAFLD). The aims of this study were to evaluate the relationship of serum iron indices and hepatic iron deposition with hepatic fibrosis or inflammation, and to assess whether the increased hepatic iron deposition is an independent predictor of progression to liver injury., Methods: The biochemical and histopathological data of thirty-nine patients with NAFLD were analyzed. Liver biopsy findings were graded according to the method described by Brunt, et al. Hepatic iron concentration was available in 29 of 39 patients., Results: The mean hepatic iron concentration and hepatic iron indices were 1,349+/-1,188 microg/g dry weight and 0.9+/-0.7 microg/g/age. Serum ferritin and body mass indices were associated with hepatic inflammation (p=0.001, p=0.006) and fibrosis (p=0.005, p=0.013). Hepatic iron concentration and hepatic iron index were not associated with hepatic inflammation and fibrosis. Multivariate analysis did not identify serum ferritin or body mass index as an independent predictor of liver injury., Conclusions: Hepatic iron deposition shows no association with the degree of hepatic inflammation or fibrosis. Hepatic iron is not an independent predictor of hepatic injury in patients with NAFLD.

Schwannomas are benign nerve sheath tumors that originate from any anatomical site. Most schwannomas occur in the head, neck or limbs, but rarely occur in the retroperitoneal space. Furthermore, the schwannoma originating from the vagus nerve of retroperitoneal space is much rare. We experienced a case of retroperitoneal schwannoma of the vagus nerve. A 34-year-old male was referred to our hospital for the evaluation of abdominal mass on ultrasonography. Endoscopic examination revealed submucosal tumor-like lesion on high body of the stomach. Computed tomography (CT) revealed that the stomach was compressed by a solid tumor in the retroperitoneum. On exploratory laparotomy, this mass turned out to be a baseball sized mass in the retroperitoneal space. The mass was excised in an encapsulated state. Histological examination with immunohistochemical stains revealed a schwannoma of the vagus nerve.

Crohn's disease is a chronic inflammatory bowel disease which affects mainly children and young adults, and its cause remains unknown so far. Infliximab, a monoclonal antibody to the pivotal cytokine tumor necrosis factor-alpha, has been approved as a drug for both induction and maintenance therapy for moderately to severely active, or fistula-complicated Crohn's disease. The authors report a 12-year-old male patient diagnosed as Crohn's disease complicated with a perianal fistula, which was refractory to the conventional therapy. After the 0, 2, and 6 week scheduled intravenous infusion of infliximab, the patient reached to clinical remission in both subjective symptoms and objective manifestations. For children or young adults who develop Crohn's disease in a refractory course, infliximab may serve as a drug which leads to a clinical improvement or even to an extent of remission.

Spontaneous perforation of the common bile duct (CBD) is a rare event in adults. Most cases of CBD perforation are iatrogenic after invasive procedures such as endoscopic retrograde cholangiopancreatography (ERCP) or cholecystectomy. We report a case of an 81-year-old woman who presented with severe right upper abdominal pain, fever, and chills. Abdominal CT showed multiple gallbladder and CBD stones and loculated fluid collection in the inferoposterior portion of the stomach. ERCP showed the leakage of contrast media into the peritoneal cavity from the CBD. We performed endoscopic sphincterotomy (EST) and endoscopic nasobiliary drainage (ENBD) to decompress the CBD instead of emergent surgical intervention. One week later, cholangiography via ENBD tube revealed that there was no more leakage of the contrast media from the CBD. We performed cholecystectomy, removal of the CBD stones after exploration of the CBD, and T tube insertion. The perforated site of the CBD was closed and there was no more fluid collection in the inferoposterior portion of the stomach. Medical treatment including endoscopic procedures was useful for healing of the perforated CBD.

Background/aims: Nonalcoholic fatty liver disease (NAFLD) represents a spectrum of conditions that are mainly characterized histologically by macrovesicular hepatic steatosis. There are two histologic patterns of NAFLD: simple steatosis alone and steatohepatitis. The factors leading from simple steatosis to nonalcoholic steatohepatitis (NASH) are still obscure. The datas from several studies have suggested that leptin could be involved in the progression from hepatic steatosis to steatohepatitis including the fibrosis. We evaluated serum leptin levels in patients with NAFLD to determine whether any relationships existed between the leptin levels and the severity of hepatic inflammation or fibrosis., Methods: We studied 62 patients with NAFLD who were diagnosed at the Hallym University Sacred Heart Hospital from July 2001 to May 2004. We measured the serum leptin level in all cases and liver biopsy samples were obtained from 31 cases. The liver biopsy specimens were graded according to methods described by Brunt. Spearman rank correlations were used to detect the associations between the serum leptin and the various anthropometric and biochemical variables. The relationship between the histologic severity and the serum leptin level was evaluated with logistic regression analysis., Results: Serum leptin levels correlated with insulin, c-peptide, ALT and homeostasis model assessment insulin resistance, but not with BMI, age and gender. Serum leptin level also correlated with hepatic fibrosis, but not with hepatic steatosis or inflammation. However, the serum leptin level was not a significant independent predictor of the grade of hepatic steatosis, inflammation and fibrosis on the univariate analysis., Conclusions: The serum leptin level was not an independent predictor of the severity of liver damage in NAFLD.

Background/aims: Nonalcoholic fatty liver disease (NAFLD) has been characterized by a wide spectrum of liver damages that span from steatosis to cryptogenic liver cirrhosis and even to hepatocellular carcinoma (HCC). The aims of this study were to determine whether the prevalence of HCC arising from cryptogenic cirrhosis has increased during the last ten years and to characterize the clinical features of cryptogenic HCC in Korea., Methods: A retrospective and hospital-based analysis of the clinical data was done in 1,145 HCC patients; group A (Jan. 1993-Dec. 1995), group B (Jan. 2000-Dec. 2002). The etiologies of HCC with liver cirrhosis in group A and group B were analyzed. The risk factors of NAFLD such as obesity, type 2 diabetes mellitus, hypertriglyceridemia and hypertension between cryptogenic HCC and HCC with well-defined etiologies were compared., Results: The major leading causes of HCC in each group were hepatitis B virus infection, followed by alcohol, hepatitis C virus and cryptogenic. There was a significant increase in the proportion of cryptogenic HCC in group B (A: 2.3%, B: 5.4%, p < 0.05). In the case of HCV, it was 5.3% in group A and 9.9% in group B (p < 0.05). Although the prevalence of cyptogenic HCC was significantly increased at an interval of seven years apart, there was no significant difference in the proportions of risk factors of NAFLD between cryptogenic HCC group and well-defined etiology group., Conclusions: The prevalence of cryptogenic HCC was significantly increased in Korea during the last decade. Although statistically insignificant, there was a trend toward the higher proportion of risk factors with NAFLD in patients with cryptogenic HCC. This suggests that increased proportion of risk factors associated for NAFLD may have contributed to the development of cryptogenic HCC.

Porphyria cutanea tarda (PCT) is a disease characterized by an early appearance of severe photosensitivity with an increase in urinary uroporphyrin excretion and other porphyrins, high isocoproporphyrin fecal levels and an accumulation of protoporphyrin in erythrocyte. This disease has been known to be caused by a dramatic decrease in the activity of the uroporphyrinogen decarboxylase. Multiple infectious and environmental factors may contribute to PCT by influencing oxidative stress in the hepatocytes. We report here on a case of porphyria cutanea tarda that presented with jaundice and photosensitivity.

GIST is a rare neoplasm, the majority of GISTs are located in the stomach and small intestine. Most GISTs are diagnosed histopathologically after resection because of submucosal location. A 37-year-old female patient presented with a 2-weeks history of generalized weakness, nausea accompanied by intermittent passage of black, tarry stools. Esophagogastroduodenoscopy and ERCP showed a large round mass measuring 5 cm in diameter in the ampulla of Vater with ulcer crack. Endoscopic multiple biopsies from the mass including ulcer base were taken. Light microscopic findings showed spindle-shaped and epitheloid tumor cells having high cellularity and frequent mitotic figures. On immunohistochemical stainings, the tumor cells were positive for CD34 and smooth muscle actin. Based on these preoperative findings, a diagnosis of malignant GIST of the ampulla of Vater was made probably. After operation, immunohistochemical studies revealed positive reaction for c-kit and vimentin, as well as focally reactive for CD34 and smooth muscle actin. We report a case of GIST in the ampulla of Vater presenting with melena that was diagnosed preoperatively and postoperatively.

Hypereosinophilic syndrome is characterized by persistent blood eosinophilia of 1,500/mm(3) or more in the absence of known causes and multiorgan dysfunction by eosinophil-related tissue damage. In Korea, some cases of hypereosinophilic syndrome with hepatic involvement have been described with prolonged benign clinical courses. Most of them were diffuse or multifocal lesions in imaging studies, and any case presenting as a large single mass lesion has not been described. Herein we report a case of hypereosinophilic syndrome with hepatic involvement in a 48-year-old woman who presented with a giant single mass. By abdominal CT scan, a lobulated well-margined heterogenous mass lesion was detected in the left lateral segment of the liver. Liver biopsy revealed severe eosinophilic infiltration and centrilobular necrosis of hepatocytes. The lesion completely disappeared after steroid administration for eleven months.

Autoimmune hepatitis (AIH) is a chronic necroinflammatory liver disease of unknown cause associated with circulating autoantibodies and high serum globulin level. Systemic lupus erythematosus (SLE) is a disease of unknown etiology in which tissues and cells are damaged by pathogenic autoantibodies and immune complex, affecting multiple organs including the liver, kidney, and CNS. The difference between the hepatic involvement of SLE and autoimmune hepatitis has not been clearly defined in the past due to similarities in clinical and biochemical features. A scoring system for the diagnosis of AIH has been established, and AIH and SLE-associated hepatitis have been defined as two different entities, although both have the same autoimmune features such as polyarthralgia, hypergammaglobulinemia and circulating autoantibodies. AIH has been considered to occur infrequently in SLE. We report two cases of AIH which simultaneously satisfied the criteria of SLE.

Primary hepatic leiomyosarcoma is a very rare malignant tumor of the liver and rather slowly progressing disease. When the tumor is solitary or even multiple but located in one lobe, the treatment of choice is an operation. We report a case of huge leiomyosarcoma in a 58-year old female patient treated by surgical resection.