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Desmoid fibromatosis is a rare benign tumor, but due to its rarity and diverse clinical course, treatment guidelines have not been established. However, since a good prognosis can be expected, an accurate diagnosis and appropriate treatment are required. We describe a rare case of desmoid fibromatosis on young female that presented as huge abdominal mass.A 28-year-old female with left upper abdominal pain 1 month ago was referred.Abdominal computed tomography and magnetic resonance imaging revealed a heterogeneous soft tissue mass approximately 29 × 17 cm in size in the left abdomen with abdominal wall invasion and pathological fracture in costochondral junction of the left 8th to 10th ribs.Surgical resection was performed.33 × 23 × 6 cm sized tumorous mass showed proliferation of bland fibromatosis and myofibroblast with nuclear β-catenin expression on pathological examination. Desmoid fibromatosis arising from intra-abdominal soft tissue with ribs and pericardium invasion was diagnosed.The mainstay of treatment of symptomatic desmoid fibromatosis is surgical resection, and in the case of abdominal tumor, it can be more dangerous when it invades adjacent organ. We report a case that required additionally multidisciplinary approach for surgery and postoperative treatment of huge abdominal desmoid tumor which infiltrate bone and pericardium beyond abdominal cavity.

The objective of our study was to develop a decision tree model for the early prediction of the severity of acute pancreatitis (AP) using clinical and radiologic scoring systems.For this retrospective study, 192 patients with AP who underwent CT 72 hours or less after symptom onset were divided into two cohorts: a training cohort (n = 115) and a validation cohort (n = 77). Univariate analysis was performed to identify significant parameters for the prediction of severe AP in the training cohort. For early prediction of disease severity, a classification tree analysis (CTA) model was constructed using significant scoring systems shown by univariate analysis. To assess the diagnostic performance of the model, we compared the area under the ROC curve (AUC) with each selected single parameter. We also evaluated the diagnostic performance in the validation cohort.The Acute Physiology and Chronic Health Evaluation (APACHE)-II score, bedside index for severity in acute pancreatitis (BISAP) score, extrapancreatic inflammation on CT (EPIC) score, and Balthazar grade were included in the CTA model. In the training cohort, our CTA model showed a trend of a higher AUC (0.853) than the AUC of each single parameter (APACHE-II score, 0.835; BISAP score, 0.842; EPIC score, 0.739; Balthazar grade, 0.700) (all, p0.0125) while achieving specificity (100%) higher than and accuracy (94.8%) comparable to each single parameter (both, p0.0125). In the validation cohort, the CTA model achieved diagnostic performance similar to the training cohort with an AUC of 0.833.Our CTA model consisted of clinical (i.e., APACHE-II and BISAP scores) and radiologic (i.e., Balthazar grade and EPIC score) scoring systems and may be useful for the early prediction of the severity of AP and identification of high-risk patients who require close surveillance.

We evaluated the diagnostic performance of ultrasonography (US) plus superb microvascular imaging (SMI) compared with conventional US alone for diagnosing acute cholecystitis. We included 54 patients with suspected biliary disease. The SMI pixel count showing flow signal was measured in the region of interest of the gallbladder bed of the liver. Two radiologists independently evaluated imaging features and rated five-point diagnostic likelihood level before versus after the additional SMI using the cutoff SMI pixel count. The SMI pixel count was significantly higher in acute than in non-acute cholecystitis (169.84 vs. 27.48, p < 0.001). The optimal SMI cutoff pixel count for predicting acute cholecystitis obtained by receiver operating characteristic curve was 56.67(82.8% sensitivity, 92.0% specificity). The area under the curve value was significantly higher after the additional SMI than before (0.798-0.863 vs. 0.701-0.736, p < 0.05). US plus SMI could objectively improve diagnostic performance compared with conventional US for acute cholecystitis.

The development of common bile duct (CBD) stones after laparoscopic cholecystectomy (LC) could be a stressful event for surgeons and patients. The purpose of this study was to investigate the risk factors for and the time of occurrence of CBD stones, which are detected at a certain period after LC in patients who have no history of having CBD stone before operation. A total of 1938 patients who underwent LC for benign gallbladder lesion were retrospectively analyzed. The patients were categorized into two groups according to the development of CBD stones at least 6 months after LC (case group, control group). The risk factors for and the time of development of CBD stones after LC were evaluated. In a univariate analysis, the significant factors for the development of CBD stones were old age, acute cholecystitis, the presence of periampullary diverticulum, and the presence of gall bladder stones sized

Background/Aims In some cases, chronic diarrhea is unexplained, and small bowel disorders may be one of the causes. The aim of this study was to assess the diagnostic yield and clinical impact of video capsule endoscopy (VCE) in patients with chronic diarrhea. Methods We retrospectively analyzed records from October 2002 to August 2013 in the VCE nationwide database registry (n=2,964). Ninety-one patients from 15 medical centers (60 males and 31 females; mean age, 47±19 years) were evaluated for VCE as a result of chronic diarrhea. Results The duration of chronic diarrhea was 8.3±14.7 months. The positive diagnostic yield of VCE was 42.9% (39/91). However, 15.4% (14/91) exhibited an inconsistent result, and 41.8% (38/91) were negative. Abnormal findings consistent with chronic diarrhea included erosions/aphthous ulcers (19.8%), ulcers (17.6%), mucosal erythema (3.3%), edema (1.1%), and luminal narrowing (1.1%). The most common diagnoses were functional diarrhea associated with irritable bowel syndrome in 37 patients (40.7%) and Crohn’s disease in 18 patients (19.8%). After VCE examination, the diagnosis was changed in 34.1% of the patients (31/91). Hematochezia (odds ratio [OR], 8.802; 95% confidence interval [CI], 2.126 to 36.441) and hypoalbuminemia (OR, 4.811; 95% CI, 1.241 to 18.655) are predictive factors of a positive diagnostic yield. Conclusions VCE had a favorable diagnostic yield and clinical impact on the management of patients with chronic diarrhea.

Purpose To determine the prevalence of and investigate the risk factors for gallbladder (GB) polypoid lesions in a healthy population. Materials and methods A total of 23827 subjects who underwent abdominal ultrasonography in conjunction with health screening examinations were retrospectively analyzed. The prevalence of risk factors for GB polypoid lesions were evaluated. In addition, risk factors according to the number of polypoid lesions and the presence of stones with polypoid lesions were investigated. To analyze these risk factors, a control group was established with a 1:2 ratio matched for age and sex. Results The prevalence of GB polypoid lesions was identified as 9.96%. On multivariate analysis, chronic hepatitis B infection (CHB) and the presence of metabolic syndrome (MS) were risk factors for GB polypoid lesions. CHB and MS were also significant independent risk factors for multiple GB polypoid lesions when compared with solitary GB polypoid lesions. In addition, gastric Helicobacter pylori infection and MS were significant risk factors for GB polypoid lesions with stones when compared with GB polypoid lesions without stones. Conclusion The prevalence of GB polypoid lesions in a healthy Korean population was 9.96%. Patients with CHB and MS need to be carefully examined for such lesions.

Background/aims Acute pancreatitis is a common complication of endoscopic retrograde cholangiopancreatography (ERCP). Combination therapy w ith ora l udenafil and aceclofenac may reduce the occurrence of post-ERCP pancreatitis by targeting different pathophysiological mechanisms. We investigated whether combining udenafil and aceclofenac reduced the rates of post-ERCP pancreatitis. Methods A prospective, randomized, double-blind, placebo-controlled, multicenter study was conducted in four academic medical centers. Between January 2012 and June 2013, a total of 216 patients who underwent ERCP were analyzed for the occurrence of post-ERCP pancreatitis. Patients were determined to be at high risk for pancreatitis based on validated patient and procedure-related risk factors. Results Demographic features, indications for ERCP, and therapeutic procedures were similar in each group. There were no significant differences in the rate (15.8% [17/107] vs. 16.5% [18/109], p = 0.901) and severity of post-ERCP pancreatitis between the udenafil/aceclofenac and placebo groups. One patient in each group developed severe pancreatitis. Multivariate analyses indicated that suspected dysfunction of the sphincter of Oddi and endoscopic papillary balloon dilation without sphincterotomy were associated with post-ERCP pancreatitis. Conclusions Combination therapy with udenafil and aceclofenac is not effective for the prevention of post-ERCP pancreatitis.

Biliary decompression with antibiotic therapy is the mainstay treatment for acute cholangitis with bacteremia. A few studies have been conducted to investigate the optimal duration and route of antibiotic therapy in biliary tract infection with bacteremia. Patients with acute cholangitis with bacteremia who achieved successful biliary drainage were randomly assigned to an early oral antibiotic switch group (group A, n = 29) and a conventional intravenous antibiotics group (group B, n = 30). Patients were discharged when they were afebrile over 2 days after oral antibiotic switch and showed consecutive improvement in the laboratory index. They were followed up and assessed at 30 days after diagnosis to evaluate the eradication of bacteria, recurrence of acute cholangitis, and 30-day mortality rate. There were no statistically significant differences between the two groups in baseline characteristics, clinical and laboratory index, severity of acute cholangitis, bacteria isolated from blood cultures, and clinical outcomes. The rate of eradication of bacteria was 93.1 % in group A and 93.3 % in group B, respectively (p = 0.97). Using non-inferiority tests, the rate of eradication of bacteria in group A was not inferior to that in group B (95 % CI −0.13 to 0.14, p = 0.97). There was no statistically significant difference in the recurrence of acute cholangitis and a 30-day mortality rate between the two groups. Early switch to oral antibiotic therapy following adequate biliary drainage for treatment of acute cholangitis with bacteremia was not inferior to conventional 10-day intravenous antibiotic therapy.

Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. LCDD is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have been few reports of isolated LCDD. We report a rare case of LCDD limited to a duodenal polyp. A 63-yr-old man visited our hospital for health screening without symptoms in 2009. On gastrofiberscopy, a duodenal polyp was observed. The biopsy showed diffuse infiltration by atypical plasma cells, which were positive for kappa-type light chains by immunohistochemistry. While the patient refused further management, we could find no evidence of recurrence until 2 yr after the initial diagnosis. It has been reported that isolated LCDD has relatively good prognosis compared to systemic LCDD. However, treatment for this disease has not been established yet.

Background Udenafil, a phosphodiesterase-5 inhibitor, may decrease sphincter of Oddi tone and allow efficient cannulation. Objective To determine whether prophylactic udenafil reduces the rates of occurrence of post-ERCP pancreatitis. Design Prospective, randomized, double-blind, placebo-controlled, multicenter study. Setting Three academic medical centers. Patients From November 2008 to November 2010, a total of 278 patients who underwent ERCP were analyzed. Intervention ERCP. Main Outcome Measurement Rate of post-ERCP pancreatitis. Results Demographic features, indications for ERCP, and therapeutic procedures were similar in each group. The overall rate of pancreatitis was 7.9% (22/278). There was no significant difference in the rate (8.0% [11/137] vs 7.8% [11/141], P = .944) and severity of post-ERCP pancreatitis between the udenafil and placebo groups. Severe pancreatitis developed in 1 patient in the placebo group. On both univariate and multivariate analyses, age 40 years or younger, suspected sphincter of Oddi dysfunction, complete pancreatic duct opacification, and failed cannulation were associated with post-ERCP pancreatitis. Only mild udenafil-related complications occurred, including flushing (n = 3) and headache (n = 3). Limitations Unselected patient group, overestimation of the rate of pancreatitis in the placebo group. Conclusion Udenafil was not effective for prevention of post-ERCP pancreatitis in this study. (Clinical trial registration number: KCT0000021.)

Endoscopic retrograde cholangiopancreatography (ERCP) has been increasingly performed in the elderly patients, yet little is known concerning objective criteria of safety. This study aimed to determine the potential predictors for the procedure-related outcomes.Two hundred eighty-one patients older than 70 years who were indicated for ERCP (group A [n = 195], 70-79 years of age; group B [n = 86], ≥ 80 years of age) were prospectively enrolled and analyzed for the development of serious adverse events related to ERCP.ERCP was not performed in six patients at high risk for the procedure. There were significant differences between group A and B in Duke Activity Status Index (DASI) (23.1 vs 14.9, P 0.01) and Eastern Cooperative Oncology Group performance status (3 and 4, 49/195 vs 33/86, P 0.05). Major ERCP-related complications (hypotension, severe bradycardia, hypoxia, myocardial infarction, cerebral infarction) occurred in five patients from group B and three from group A. Post-ERCP pancreatitis occurred in one patient from group A and bleeding in one from group B. In univariate analysis, old age (≥ 80 years), American Society of Anesthesiologists score ≥ 3, and DASI 10 were statistically significant predictors for overall serious events related to ERCP. In the multivariate analysis, DASI 10 (only manage to ambulate) was independent predictor for overall serious events related to ERCP.DASI score is useful predictor for the feasibility assessment of safe ERCP in the elderly patients.

We aimed to determine the prevalence of cholesterol gallstones, the compositional changes of gallstones and its predisposing factors in Korea with this single-center study.Data of 365 patients who underwent cholecystectomy for cholecystolithiasis from July 2008 to September 2011 were reviewed. Based on the compositional analysis of the gallstones, patients were assigned to either cholesterol gallstone group or pigment gallstone group. The characteristics of the patients and the gallstones were summarized and compared.After eight patients with mixed gallstones were excluded, 357 patients were enrolled in the study, including cholesterol gallstones in 175 (49.0%) and pigment gallstones in 182 (51.0%). The number of patients with cholecystolithiasis increased but the prevalence of cholesterol gallstone decreased with age. Compared with the pigment gallstone group, the cholesterol gallstone group was associated with young age (50 years), female gender, obesity (body mass index ≥ 25 kg/m²) and the absence of chronic liver diseases.The relative prevalence of cholesterol gallstones in Korea has been stationary so far, but may change in the future since cholesterol gallstones are increasingly prevalent in the young generation.

Background: Entecavir (ETV) has potent antiviral activity against hepatitis B virus (HBV), and the emergence of drug resistance is rare in nucleoside-naive patients. Resistance requires simultaneous appearance of three mutations which account for the very low resistance profile of ETV. We experienced one case of genotypic ETV resistance with viral rebound during ETV treatment of nucleoside-naive patients with chronic hepatitis B (CHB). Case: A 50-year-old HBV e antigen-positive man received ETV 0.5 mg/day for 120 weeks. The level of HBV DNA was 9.0 log10 copies/ml at baseline, declined to a nadir of 2.7 at week 60 and then rebounded to 6.0 at week 108 and 7.5 at week 120. The serum alanine aminotransferase level did not increase during ETV treatment. The ETV resistance-related substitution (T184A) and lamivudine resistance-related substitutions (L180M and M204V) were detected by sequence analysis at week 96. Conclusions: The three substitutions associated with ETV and lamivudine resistance developed simultaneously without complete suppression in a nucleoside-naive CHB patient after extended therapy. In spite of the extremely rare chance of viral mutation during ETV treatment, treatment-naive patients with high pretreatment viral loads and detectable HBV DNA during treatment should be carefully monitored or undergo targeted surveillance for resistance.

A 68-year-old woman presented with yellowish discharge oozing from a fistula opening in the upper epigastric area that had persisted for one month prior to her visit. The patient had undergone a left lateral segmentectomy of the liver ten years prior for treatment of intrahepatic duct (IHD) stones. An abdominal computed tomography (CT) scan showed focal stricture and proximal dilatation of remnant IHD and a 1 cm-sized rim-enhancing lesion located under the surgical bed of the abdominal wall surrounding the dilated remnant IHD. Despite conservative management including nasobiliary drainage, no further improvement was anticipated. Partial hepatectomy and fistulectomy were performed for pathologic diagnosis and treatment of the enhancing lesion. Histopathology revealed adenocarcinoma. In this case, cholangiocarcinoma might have arisen in association with IHD stones and then developed a choledocho-cutaneous fistula as a clinical manifestation.

We report a rare case of hypereosinophilic syndrome (HES) presenting with intractable gastric ulcers. A 71-year-old man was admitted with epigastric pain. Initial endoscopic findings revealed multiple, active gastric ulcers in the gastric antrum. He underwent Helicobacter pylori (H pylori) eradication therapy followed by proton pump inhibitor (PPI) therapy. However, follow-up endoscopy at 4, 6, 10 and 14 mo revealed persistent multiple gastric ulcers without significant improvement. The proportion of his eosinophil count increased to 43% (total count: 7903/mm(3)). Abdominal-pelvic and chest computed tomography scans showed multiple small nodules in the liver and both lungs. The endoscopic biopsy specimen taken from the gastric antrum revealed prominent eosinophilic infiltration, and the liver biopsy specimen also showed eosinophilic infiltration in the portal tract and sinusoid. A bone marrow biopsy disclosed eosinophilic hyperplasia as well as increased cellularity of 70%. The patient was finally diagnosed with HES involving the stomach, liver, lung, and bone marrow. When gastric ulcers do not improve despite H pylori eradication and prolonged PPI therapy, infiltrative gastric disorders such as HES should be considered.

The aim of this study was to evaluate the changes in prevalence and the related factors of Helicobacter pylori (H. pylori) infection in Korean health check-up subjects during the period of 8 years.Among 89,231 subjects who visited the Health Promotion Centers of Severance hospital or Chung-Ang University hospital from Jan. 1998 through Dec. 2005, a total of 10,553 subjects who received esophagogastroduodenoscopy (EGD) and H. pylori test were enrolled. H. pylori infection was assessed by histologic examination. Changes of the prevalence of H. pylori infection during 8 years, and infection-related factors such as demographic characteristics, body mass index, ABO blood types, endoscopic findings (presence of peptic ulcer diseases), educational level, economic status, smoking habits, and alcohol intake in year 2005 were analyzed.The mean age of 10,553 subjects (7,329 men, 3,224 women) was 49.7+/-10.4 years (range from 17 to 92 years). The prevalence of H. pylori infection at the first, second, third, fourth, fifth, sixth, seventh, and eighth year were 64.7%, 58.1%, 54.2%, 50.4%, 48.9%, 49.5%, 39.6%, and 40.0%, respectively, and these serial decreases in prevalence over 8 years were statistically significant (p0.001). Regardless of sex, age or EGD findings, the prevalence of H. pylori infection was significantly decreased. In the analysis of the H. pylori infection-related factors in 2005, only age and EGD findings (peptic ulcer diseases) were significant factors.The prevalence of H. pylori infection was significantly decreased during 8-year period in Korean health check-up subjects. Age and peptic ulcer diseases were the two significant factors related to H. pylori infection in Korea.

The cavernous sinus of skull base is a extremely rare metastastatic site for hepatocellular carcinoma (HCC). A 51-year-old man was diagnosed with HCC by liver biopsy and palliative radiotherapy on HCC including main portal vein was performed. One month later, he was admitted due to sudden onset ptosis. Neurologic findings were normal except for abnormal movement of right eye, and it raised the possibility of abnormality in the right occulomotor, trochlear and the abducens nerves. Contrast-enhanced CT scan of brain showed a mass with homogeneous enhancement involving the right cavernous sinus. T2-weighted axial MR images demonstrated a homogeneous mass with intermediate signal intensity, and contrast-enhanced axial T1-weighted MR images demonstrated a mass with homogeneous enhancement in the right cavernous sinus. We describe a case of HCC metastasis to the cavernous sinus with symptoms of ptosis and disturbance of right eyeball movement.

Burkitt's lymphoma is a rare disease that belongs to the aggressive non-Hodgkin's lymphoma. Herein, we report a case of primary hepatic Burkitt's lymphoma. A 19-year-old man visited the hospital for right upper quadrant pain. He felt fatigue for two months. Physical examination revealed hepatomegaly and no palpable lymph node. He had no fever, weight loss, or night sweating. Laboratory finding showed mild anemia (hemoglobin, 12.4 g/dL), mild elevated transaminase (ALT, 52 IU/L), elevated lactate dehydrogenase (LDH, 437 IU/L), and alkaline phosphatase (ALP, 129 IU/L). The viral marker was positive for HBsAg, HBeAg, anti-HBs, and anti-HBc (IgG), and negative for anti-HBe, anti-HCV, and anti-HIV. CEA, AFP, and CA19-9 levels were within normal ranges. The HBV DNA quantitation was 1.3 x 10(9) copies/ml. Abdominal-Pelvis CT scan and abdominal MRI finding were compatible with malignant lymphoma. Liver biopsy examination confirmed Burkitt's lymphoma. No metastasis was detected in the thoracic cavity, bone marrow, and spinal fluid. The patient was treated with the combination regimen of cyclophosphamide, doxorubicin, vincristine, prednisone and high dose methotrexate. Cytosine arabinoside and methotrexate were added for CNS prophylaxis by intrathecal installation. Chemotherapy was administered every 3 weeks for fifteen cycles. Serial follow-up CT scan showed a marked decrease in the size of hepatic lesions. Follow-up CT scan and PET-CT scan were performed 4 weeks after the final cycle disclosed no definite residual or active lesion confirming the state of complete remission.

Capsule endoscopy can detect lesions associated with Crohn's disease in the small bowel that go unrecognized by conventional radiologic imaging. The aim of this study was to assess the value of capsule endoscopy in the classification of patients with Crohn's disease. This study was carried out on 52 patients with known Crohn's disease. Small bowel follow-through findings were compared with capsule endoscopic findings. All patients were classified according to the extent of the disease: small bowel-colon type, small bowel type, or colon type. Seventeen patients who were reported as being normal showed evidence of small bowel Crohn's disease according to capsule endoscopy. Differences were observed between the small bowel follow-through and the capsule endoscopy classification (kappa=0.54). Treatment was changed based on the results of wireless capsule endoscopy in 15 patients.

In order to identify microsatellite instability (MSI), the test based on the polymerase chain reaction (PCR) can be used. However, PCR is not routinely performed in all hospital laboratories. Recently, immunohistochemistry (IHC) for MLH1 and MSH2 proteins has been reported as a rapid and useful method for MSI. However, the efficacy of IHC in the detection of the MSI has not been well established. The aim of this study was to evaluate the usefulness of IHC in the detection of the MSI by comparing it with the test results using PCR in colorectal cancer (CRC).Paraffin-embedded normal and tumor tissues from seventy-five patients who underwent surgical resection of CRC were used. Abnormal expression of MLH1 and MSH2 protein was determined by IHC using MLH1 and MSH2 antibodies. Normal and tumor DNAs were obtained from thirty CRC tissues that showed abnormal expression of MLH1 and MSH2 proteins by IHC. The MSI status was confirmed by PCR using five markers.Thirty tumors showed abnormal expression of MLH1 and MSH2 proteins by IHC, but only three tumors out of them were confirmed to have MSI by PCR.This result suggests that IHC with MLH1 and MSH2 antibodies does not seem to be a useful method to identify MSI in CRC, therefore PCR is required for detection of the MSI.

Treatment of chronic hepatitis B with interferon results in a sustained loss of hepatitis B virus DNA and hepatitis B e antigen (HBeAg) and remission of liver disease only in a proportion of cases. Recently, mutations of hepatitis B virus (HBV) core gene have been reported as being related to the failure of interferon treatment in chronic hepatitis B. This study investigated whether core gene mutations of HBV are related to non-response to interferon therapy and whether the recurrence of HBeAg and HBV DNA in initial responders to interferon therapy is associated with the emergence of HBV core gene mutants.The precore/core gene sequence was determined by polymerase chain reaction (PCR) and direct sequencing of PCR product in serum samples obtained before interferon treatment from 10 responders and 10 non-responders to interferon therapy. In addition, precore/core gene sequence was determined in serum samples obtained before interferon treatment and after recurrence from 10 patients who showed recurrence of HBeAg and HBV DNA after initial response to interferon therapy.In samples from 10 responders, there were 7 missense mutations and 71 silent mutations. However, there were 43 missense mutations and 109 silent mutations in samples from 10 non-responders. In samples obtained before interferon treatment from the 10 patients who showed recurrence after initial response, 8 missense mutations and 74 silents mutations were found. The nucleotide sequences from the samples obtained after the recurrence showed 6 silent nucleotide substitutions compared with the sequences from the samples obtained before interferon treatment.Mutations in the core protein of HBV occur more frequently in non-responders than responders to interferon therapy of chronic hepatitis B and may be a factor responsible for the failure of interferon treatment. The recurrence of HBeAg and HBV-DNA in initial responders to interferon therapy is not associated with the emergence of the HBV core gene mutants.