BACKGROUND: One of the most frequent cytogenetic abnormality in acute myeloid leukemia (AML) is t(8;21) (q22;q22), with rearrangement of the AML1 gene on chromosome 21q22 and the ETO gene on chromosome 8q22. In adult AML1/ETO-associated leukemia patients, chemotherapy alone results in cure rates that are comparable to or better than those achieved with allogenic bone marrow transplantation. Despite the relatively good prognosis of AML1/ETO fusion transcript, relapse of leukemia remains the most common cause of treatment failure. Monitoring minimal residual disease (MRD) in leukemia has two main aims : to assess the effectiveness of treatment and to detect early signs of relapse. Reverse transcription-polymerase chain reaction (RT-PCR)- based methods is the rapid and sensitive method in the identification of this molecular abnormality. The purpose of this study is to ensure the usefulness of the RT-PCR technique for detecting MRD in AML1/ETO-associated leukemia patients in remission and to establish the correlation of the serial detection of AML1/ETO fusion transcripts after complete remission and long-term outcome. METHODS: From the bone marrow aspirates of 25 AML1/ETO positive AML patients, serial detection of AML1/ETO fusion trascripts was performed using RT-PCR. RESULTS: AML1/ETO fusion transcripts were positive in 14 cases who did not show t(8;21). In serial assay, AML1/ETO fusion transcripts was positive in 9 cases and negative in 13 cases at 10 weeks after complete remission. AML1/ETO fusion transcripts (+) group has 107.4+/-18.2 months suvival and AML1/ETO fusion transcripts (-) group has 47.3+/-18.0 months survival. However, there is no significance (P=0.11). CONCLUSION: This study suggests that the early negative conversion of AML1/ETO fusion transcript may be the good prognostic predictor. The RT-PCR technique is useful for detecting minimal residual disease in leukemia patients in remission and it may improve the therapeutic strategy for leukemia.
Adult ; Male ; Female ; Humans ; Bone Marrow Transplantation

BACKGROUND: Endoscopic ultrasound-guided fine needle aspiration cytology (EUS-FNAC) is currently the most commonly used procedure for obtaining cytologic specimens of the pancreas. It is accurate, minimally invasive, safe and cost-effective. However, there is discrepancy between cytological and surgical diagnoses. This study was aimed at evaluating the diagnostic accuracy of EUS-FNAC of the pancreas. METHODS: We performed a retrospective review of 191 cases of pancreatic lesions initially diagnosed by EUS-FNAC with subsequent histological diagnosis between 2010 and 2012 in the Department of Pathology, Seoul National University Hospital. Cytologic and surgical diagnoses were categorized into five groups: negative, benign, atypical, malignant, and insufficient for diagnosis. Subsequently, 167 cases with satisfactory yield in both surgical and cytology specimens were statistically analyzed to determine correlations with diagnosis. RESULTS: In comparison to surgical diagnoses, cytologic diagnoses were true-positive in 103 cases (61.7%), true-negative in 28 cases (16.8%), false-positive in 9 cases (5.4%), and false-negative in 27 cases (16.1%). The diagnostic accuracy was 78.4%, sensitivity was 79.2%, and specificity was 75.7%. The positive predictive value was 92.0%, and negative predictive value was 50.9%. CONCLUSIONS: EUS-FNAC has high accuracy, sensitivity, specificity and positive predictive value. Overcoming the limitations of EUS-FNAC will make it a useful and reliable diagnostic tool for accurate evaluation of pancreatic lesions.
Diagnosis ; Endoscopic Ultrasound-Guided Fine Needle Aspiration* ; Pancreas ; Pathology ; Retrospective Studies ; Sensitivity and Specificity ; Seoul

Symptomatic hepatic cysts are infrequently seen. A 82-year-old woman was admitted because of growing abdominal mass and pain. On admission, the mass was palpated on right upper quadrant of the abdomen. Ultrasonography and computed tomography disclosed a huge cystic lesion of the liver. It measured 22.5 x 19.0 x 18.0 cm and had a thick wall that was irregular. Because of the patient's symptoms and the radiologic findings, the decision was made to aspirate the cyst percutaneously under fluoroscopic guidance. Percutaneous drainage yielded approximately 3300 cc of yellow brownish fluid. A cytologic evaluation of the fluid was negative for malignant cells, and a fluid analysis was described as predominantly inflammatory in nature. Cultures revealed a growth of Klebsiella oxytoca. After drainage of the cystic fluid, we instilled contrast medium. No communication between the cyst and bile ducts was seen. Seven days later, the patient was discharged. Four months after treatment, no reaccumulated fluid was observed by ultrasonography. Ten months after treatment, the patient is healthy without abdominal discomfort. We report a case of the infected huge hepatic cyst successfully treated with fluoroscopic-uided percutaneous drainage.
Abdomen ; Aged, 80 and over ; Bile Ducts ; Drainage* ; Female ; Humans ; Klebsiella oxytoca ; Liver ; Ultrasonography

Hepatic fibrogenesis, a complex process that involves a marked accumulation of extracellular matrix components, activation of cells capable of producing matrix materials, cytokine release, and tissue remodeling, is regulated by matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs). The MMP-TIMP balance can regulate liver fibrogenesis. The aim of this study was to evaluate the expression patterns of MMPs and TIMPs during thioacetamide (TAA)-induced liver fibrogenesis. Chronic liver injury was induced with TAA (200 mg/kg i.p.) for 4 or 7 weeks in male Sprague-Dawley rats. Hepatic injury and fibrosis were assessed by hematoxylin-eosin (H&E) staining, and collagen deposition was confirmed by Sirius Red staining. The level of hepatic injury was quantified by serological analysis. The transcriptional and translational levels of alpha-smooth muscle actin (alpha-SMA), MMPs, and TIMPs in the liver were measured by Western blotting, RT-PCR, and immunohistochemistry. MMP, TIMP, and alpha-SMA were observed along fibrotic septa and portal spaces around the lobules. TAA treatment increased transcription of both MMPs and TIMPs, but only TIMPs showed increased translation. The dominant expression of TIMPs may regulate the function of MMPs to maintain liver fibrosis induced by TAA.
Animals ; Collagen/metabolism ; Extracellular Matrix/chemistry/metabolism ; *Liver Cirrhosis/chemically induced/metabolism/pathology ; Male ; Matrix Metalloproteinases/genetics/*metabolism ; Rats ; Rats, Sprague-Dawley ; Thioacetamide/*toxicity ; Tissue Inhibitor of Metalloproteinases/genetics/*metabolism

BACKGROUND: Insular thyroid carcinoma (ITC) is a relatively infrequent thyroid carcinoma that has distinctive histologic features. ITC shows an aggressive clinical course and the predominant presence of an insular component, which has been reported to be an independent factor of a poor prognosis. We retrospectively examined clinical details of the nine ITC patients, which represented 9 years of experience with ITC, and investigated the expressions of variable neuroendocrine and other immunohistochemical markers associated with well-differentiated thyroid carcinomas. METHODS: We adopted an immunohistochemical approach and studied the expressions of synaptophysin, chromogranin A, CD56, NSE, S-100, RET, PPARgamma, calcitonin, galectin-3, and thyroglobulin in formalin-fixed, paraffin embedded tissue array slides of the 9 ITC patients, and investigated clinical features. Seven cases of follicular carcinoma and 4 cases of medullary carcinoma were also included as controls. RESULTS: ITCs were positive for synaptophysin (44%, 4/9), CD56 (11%, 1/9), NSE (89%, 8/9), S100 (67%, 6/9), calcitonin (22%, 2/9), galectin-3 (78%, 7/9), and thyroglobulin (100%, 9/9), but completely negative for chromogranin A, RET, and PPARgamma. CONCLUSION: ITCs express neuroendocrine markers in variable proportions and appear not to be associated with the oncoproteins of conventional thyroid carcinomas. Notably, its differential diagnosis from medullary carcinoma is required in cases showing focal calcitonin positivity.
Calcitonin ; Carcinoma, Medullary ; Chromogranin A ; Diagnosis, Differential ; Galectin 3 ; Humans ; Oncogene Proteins ; Paraffin ; PPAR gamma ; Prognosis ; Retrospective Studies ; Synaptophysin ; Thyroglobulin ; Thyroid Gland* ; Thyroid Neoplasms

NSAIDs are now the most widely prescribed of all drugs for the therapy of a large variety of disorders including rheumatologic disorders, and so the population of patients who are at risk for adverse effects of these drugs is rapidly expanding. A number of renal and electrolyte problems have been associated with the use of NSAIDs, including alterations in glome-rular filtration rate, hyperkalemia, acute interstitial nephritis and papillary necrosis. While the use of NSAIDs has also been associated with minimal change nephrotic syndrome, this complication has almost invariably occured in association with an acute interstitial nephritis. Recently, we experienced a case of minimal change nephrotic syndrome without significant interstitial inflammation associated with the use of NSAIDs. This patient is a 64-year-old female who developed the generalized edema and about 10kg of weight gain since three days ago. She had taken the anti-inflammatory drugs for five years intermittently and started taking diclofenac sodium, 25mg orally three times a day, 10 days before admission for increasing pain in her knees. Laboratory findings disclosed the following values WBC 5,200/mm3 with only 0.6% eosinophils, total serum protein 4.0g/dL, albumin 1.2 g/dL, BUN 17mg/dL, creatinine 0.8mg/dL, sodium 140 mmol/L, potassium 4.0mmol/L, chloride 113mmol/L, total cholesterol 338mg/dL, triglyceride 203mg/dL; 24-hour protein excretion 3.6g, creatinine clearance 52.8 mL/min ; serologic tests were unremarkable. A renal biopsy revealed no abnormality except for focal mild interstitial infiltration of chronic inflammatory cells with a few atrophic tubules on light microscopy. Immunofluorescence studies showed diffuse trace mesangial deposits of IgM, and electromicroscopy revealed diffuse obliteration of the epithelial foot process and villous transformation of the epitherial cell cytoplasms without electrondense deposits. These findings were consistent with minimal-change disease. Diclofenac was discontinued on admission because of the likelihood the renal disease was drugrelated and she treated with low-dose(40-80mg/d) of furosemide. Fourteen days after stopping diclofenac, her massive edema and weight gain resolved and laboratory studies showed a 24-hour urine protein excretion of 80mg and serum albumin of 2.7g/dL. There has been no relapse for five months since then.
Anti-Inflammatory Agents, Non-Steroidal ; Biopsy ; Cholesterol ; Creatinine ; Cytoplasm ; Diclofenac* ; Edema ; Eosinophils ; Female ; Filtration ; Fluorescent Antibody Technique ; Foot ; Furosemide ; Humans ; Hyperkalemia ; Immunoglobulin M ; Inflammation ; Knee ; Microscopy ; Middle Aged ; Necrosis ; Nephritis, Interstitial ; Nephrosis, Lipoid ; Potassium ; Recurrence ; Serologic Tests ; Serum Albumin ; Sodium ; Triglycerides ; Weight Gain

Intestinal lymphangiectasia (IL) is a rare disorder, characterized by dilatation of intestinal lymphatics and leakage from ruptured lacteals to the intestinal lumen. Primary IL may be due to a congenital malformation of the lymphatic system, whereas secondary IL is caused by decreased lymph flow from thoracic ductsdue to elevated left subclavian vein pressure as a result of a preceding inflammatory or neoplastic disease. IL can present as protein-losing enteropathy with clinical manifestations of hypoproteinemia, hypoalbuminemia, edema, ascites, or pleural effusions. In very rare cases, it can present as severe intestinal bleeding. We experienced a 48-year-old woman presenting with recurring hematochezia and melena. She was diagnosed bydouble balloon enteroscopy, and surgical resection was needed to stop bleeding. In conclusion, IL can present clinically as painless chronic blood loss. If IL is locally distributed, surgical resection may be needed to control bleeding and to exclude other underlying causes in some patients.
Ascites ; Dilatation ; Double-Balloon Enteroscopy ; Edema ; Female ; Gastrointestinal Hemorrhage ; Hemorrhage ; Humans ; Hypoalbuminemia ; Hypoproteinemia ; Lymphatic System ; Melena ; Middle Aged ; Pleural Effusion ; Protein-Losing Enteropathies ; Subclavian Vein

BACKGROUND/AIMS: This study aimed to better understand gene expression profiles of human hepatic stellate cell (HSC) activation and the relationship with the Wnt signaling pathway. METHODS: The global transcript levels in platelet derived growth factor-BB (PDGF-BB)-stimulated hTERT HSCs were analyzed using oligonucleotide microarrays. Oligonucleotide microarrays with 19K human oligo chips were performed to obtain gene expression profiles associated with proliferation in human hTERT HSCs. The microarray data was verified by real time quantitative PCR and expression of the components of Wnt signaling was analyzed by Western blot. RESULTS: Microarray data showed 243 up-regulated and 265 down-regulated genes in PDGF-BB-treated HSCs. The changes in expression of glypican3 and BH3 interacting domain death agonist (BID) mRNA in real time quantitative PCR, especially among the highly up- or down-regulated genes, were statistically consistent with the microarray data. The Wnt signaling pathway components, frizzled10 (FZD10) and calcium/calmodulin-dependent protein kinase II alpha (CAMK2A), showed increased expression in the short time course microarray and the up-regulation of FZD10 also occurred at the protein level. Our data showed various gene expression profiles during activation of human HSC. CONCLUSIONS: The up-regulated expression of FZD10 and CAMK2A suggests that the Wnt/Ca2+ signaling pathway is active in hTERT HSCs and may participate in HSC activation and proliferation
Angiogenesis Inducing Agents/*pharmacology ; Blotting, Western ; Calcium-Calmodulin-Dependent Protein Kinase Type 2/genetics/metabolism ; Cell Line ; Cell Proliferation ; Frizzled Receptors/genetics/metabolism ; Gene Expression Profiling ; Hepatic Stellate Cells/cytology/*metabolism ; Humans ; Oligonucleotide Array Sequence Analysis ; Platelet-Derived Growth Factor/*pharmacology ; Polymerase Chain Reaction ; Receptors, G-Protein-Coupled/genetics/metabolism ; *Signal Transduction ; Up-Regulation ; Wnt Proteins/genetics/*metabolism

PURPOSE: Nonfunctioning endocrine tumors of the pancreas have no specific symptoms. Therefore, diagnosis is usually delayed and the malignancy rate at the time of diagnosis is reported to be higher than 50%. However, it is difficult to discriminate malignant from benign nonfunctioning endocrine tumors preoperatively. The purpose of this study was to investigate clinical characteristics of nonfunctioning endocrine tumors of the pancreas, including predictive factors of malignancy and prognostic factors affecting long-term survival. METHODS: Between 1992 and 2010, clinicopathological data of 53 patients with nonfunctioning endocrine tumors proven by surgical pathology were reviewed retrospectively. RESULTS: Of the 53 patients, mean age was 54 years-old and the male to female ratio was 1:1.2 Median follow up was 32.1 months. At the time of diagnosis, liver metastasis was detected in 4 patients. Curative resection was achieved in 49 patients, and 17% of them had recurrence, which was most common in liver. The overall 5-year survival rate was 85.1%. Both the WHO classification (p<0.001) and AJCC staging (p<0.001) correlated well with long-term survival. Univariate analysis revealed preoperative body weight loss (p<0.001), weak enhancement at the early arterial phase (p=0.043), lymph node metastasis (p<0.001), liver metastasis (p=0.001), perineural invasion (p=0.001), or lymphovascular invasion (p=0.010) as prognostic factors. CONCLUSION: Nonfunctioning endocrine tumor of the pancreas has favorable survival outcomes. Lymph node metastasis (p<0.001), liver metastasis (p=0.001), perineural invasion (p=0.001), and lymphovascular invasion (p=0.010) are poor prognostic factors.
Body Weight ; Female ; Follow-Up Studies ; Humans ; Liver ; Lymph Nodes ; Male ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Pancreas ; Pathology, Surgical ; Prognosis ; Recurrence ; Survival Rate

The terms "gastrointestinal stromal tumor" (GIST) have been applied to mesenchymal tumos that represent neither typical leiomyoma nor schwannoma. The majority of GISTs are Located in the stomach and small intestine, and only 4% of GISTs are found in duodenum. The most important characteristic is their indolent, slow-growing nature, rendering the most common definitions of malignancy invalid and inapplicable. Clinical and pathological criteria to differentiate benign from malignant GISTs are not well established. Tumor size and mitoic activity are commonly considered as important features, allowing for the prediction of biological behaviour and outcome. The case of a 87-year-old male patient who was presented with melena and acute anemia is herein reported. Esophagogastroduodenoscopy and computed tomography of the abdomen revealed two submucosal tumors in the stomach and duodenum. Histological and immunohistochemical studies on the surgical resection specimen revealed a gastrointestinal stromal tumor of an uncommiteed type, with no evidence of increased mitotic activity. The patient was treated with local excision of the tumors and is now in a favorable state.
Abdomen ; Aged, 80 and over ; Anemia ; Duodenum ; Endoscopy, Digestive System ; Gastrointestinal Stromal Tumors* ; Hemorrhage* ; Humans ; Intestine, Small ; Leiomyoma ; Male ; Melena ; Neurilemmoma ; Stomach