Gastrointestinal stromal tumors (GISTs), which are the most common mesenchymal tumors of the gastrointestinal tract, have represented an important advance in oncology field with the success of molecular targeted therapy. Since the approval of the tyrosine kinase inhibitor (imatinib) in 2002, the survival of patients with advanced GISTs has significantly increased. Accurate histopathologic diagnosis of GISTs and multidisciplinary approach has become more important for successful management of GISTs. Recently, imatinib has become a standard treatment even in adjuvant setting, and regorafenib has been approved for advanced GIST after failure of imatinib and sunitinib. This review presents here the updated results of relevant clinical studies for the further revision to the guideline of Korean GIST study group. We hope this review will help enhance the quality of diagnosis, treatment, and care of patients with GIST in Korea.
Benzamides ; Gastrointestinal Stromal Tumors ; Gastrointestinal Tract ; Humans ; Indoles ; Korea ; Molecular Targeted Therapy ; Phenylurea Compounds ; Piperazines ; Protein-Tyrosine Kinases ; Pyridines ; Pyrimidines ; Pyrroles ; Imatinib Mesylate

Gastrointestinal stromal tumors (GISTs), which are the most common mesenchymal tumors of the gastrointestinal tract, have represented an important advance in oncology field with the success of molecular targeted therapy. Since the approval of the tyrosine kinase inhibitor (imatinib) in 2002, the survival of patients with advanced GISTs has significantly increased. Accurate histopathologic diagnosis of GISTs and multidisciplinary approach has become more important for successful management of GISTs. Recently, imatinib has become a standard treatment even in adjuvant setting, and regorafenib has been approved for advanced GIST after failure of imatinib and sunitinib. This review presents here the updated results of relevant clinical studies for the further revision to the guideline of Korean GIST study group. We hope this review will help enhance the quality of diagnosis, treatment, and care of patients with GIST in Korea.
Benzamides ; Gastrointestinal Stromal Tumors ; Gastrointestinal Tract ; Humans ; Indoles ; Korea ; Molecular Targeted Therapy ; Phenylurea Compounds ; Piperazines ; Protein-Tyrosine Kinases ; Pyridines ; Pyrimidines ; Pyrroles ; Imatinib Mesylate

No abstract available.
Child* ; Humans ; Neurilemmoma* ; Neurofibromatosis 1

No abstract available.
Neuroblastoma* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma*

No abstract available.
Down Syndrome* ; Leukemia*

PURPOSE: It is not clear that the development of glomerular injury and aggravation by tumor necrosis factor alpha (TNF-alpha) is related to intrarenal or serum concentration of TNF-alpha. So, we studied the relationship between the concentration of TNF-alpha and aggravation of glomerular damage in the Henoch-Schonlein nephritis(HSN) and idiopathic nephrotic syndrome(INS). METHODS: We collected the sera and urines of 21 patients with Henoch-Schonlein purpura(HSP) and 22 patients with INS visited Chungbuk National University hospital from March 1998 to March 2001. The concentration of TNF-alpha in the sera and urines were measured by sandwich ELISA. RESULTS: Serum TNF-alpha levels in the HSP patients with renal involvement were significantly higher than those without renal involvement(P=0.009). But urine TNF-alpha levels have no correlation with renal involvement(P=0.088). In the HSN patients, proteinuria have a significant correlation with serum TNF-alpha levels(P=0.004) but less correlation with urine TNF-alpha levels(P=0.053). Otherwise, proteinuria have no correlation with serum TNF-alpha levels(P=0.763) but have a significant correlation with urine TNF-alpha levels(P=0.007) in INS. CONCLUSION: These result suggest that the serum concentration of TNF-alpha would be important to glomerular involvement in HSP. And, it is interesting that proteinuria shows a significant relation with serum TNF-alpha levels in the HSN, but with urine TNF-alpha levels in the INS. This means the major production of TNF-alpha may be originated by extrarenal inflammation in the HSN and by intrarenal tubulo-interstitial damage due to proteinuria in the INS.
Chungcheongbuk-do ; Enzyme-Linked Immunosorbent Assay ; Humans ; Inflammation ; Nephritis* ; Nephrotic Syndrome* ; Proteinuria* ; Tumor Necrosis Factor-alpha*

We analyzed the clinical and laboratory features of ten children with acute megakaryoblastic lukemia (M7)and compared the findings with those reported in the literature. The diagnosis was supprted by ultrastructural examination of platelet peroxidase or immunophenotyping for glycoprotein IIb/IIIa. Of the ten children, five were girls and five were boys. The median age at diagnosis was 13 months. Two patients had prominent myelofibrosis and one patient had Down syndrome. Nine patients were treatd with low-dose cytosine arabinoside (10mg/m2)administered intravenously, or subcutaneously, or intramuscularly, twice daily in 21 day courses. Seven patients achieved hematologic response and three patients are alive without evidence of disease. The 4 year event free survival rate was30.0%. It is our impression that the prevalence of acute megakaryoblastic leukemia has been under-estimated, and low-dose cytosine arabinoside treatment may be of value in its management. This approach may be particularily useful in hospitals with scarce well-equipped facilities, since this protocol does not induce profound marrow hypoplasia and intensive supportive measures are not required as they would be with the use of more aggressive drug combination.
Blood Platelets ; Bone Marrow ; Child* ; Cytarabine ; Diagnosis ; Disease-Free Survival ; Down Syndrome ; Female ; Glycoproteins ; Humans ; Immunophenotyping ; Leukemia, Megakaryoblastic, Acute* ; Megakaryocyte Progenitor Cells ; Peroxidase ; Prevalence ; Primary Myelofibrosis

BACKGROUNDS: Pancreaticoduodenectomy has been applied to diseases of periampullary region since 1935 by Whipple and associates, but this procedure is so complexed that associated with high morbidity and mortality. In the treatment of periampullary cancer, curative surgical resection only promises the possibilities of cure, because other methods of treatment including chemotherapy and radiation therapy have been of little or no benefit to improve the survival rate. METHODS: We performed the clinical analysis on 123 cases of pancreaticoduodenectomies at the department of Surgery, Chonnam University Hospital, during the past 11 years from June 1986 to May 1997 to assess the associated factors with postoperative complications and the survival rate. RESULTS: Among 123 cases, 104 cases(84.6%) were malignant tumor of periampullary region, 16 cases were benign lesion, and 3 cases were type IV pancreatic injury. Pancreaticoduodenectomy was performed in 115 cases, pylorus-preserving pancreaticoduodenectomy in 4 cases, total pancreatectomy in 2 cases and extended pancreaticoduodenectomy in 2 cases. Postoperative complications were developed in 28 cases(22.8%) and the major cause of postoperative complication was the anastomotic leakage, especially in the pancreatico-jejunostomy site. The overall operative and hospital mortality rate was 7.3%. Preoperative percutaneous transhepatic biliary drainage(PTBD) and the serum level of albumin and bilirubin were not related to the development of postoperative complications. Factors affecting survival after resection of malignant tumor were the size of tumor, presence of lymph node metastasis and tumor infiltration to adjacent tissue, but the level of CEA, alphaFP, and CA 19-9 and tumor differentiation were not related to the survival rate statistically. CONCLUSION: Optimal preoperative preparation of the patient and a meticulous and standarized operative technique are mandatory to minimize the operative morbidity and mortality after pancreaticoduodenectomy. In the treatment of periampullary cancer, radical surgical resection is recommended for long-term survival, as well as the development of other treatment modalities to prevent the postoperative recurrence.
Anastomotic Leak ; Bilirubin ; Drug Therapy ; Hospital Mortality ; Humans ; Jeollanam-do ; Lymph Nodes ; Mortality ; Neoplasm Metastasis ; Pancreatectomy ; Pancreaticoduodenectomy* ; Postoperative Complications ; Recurrence ; Survival Rate

A totally implanted venous access system was inserted in 19 children with cancer. The devices were utilized for the administration of antineoplastic drugs, parenteral fluids, antibiotics, and blood products. Total duration of implantation was 4,046 days for 23 implanted system (range 7-445 days). Complications included cather infection (0.247/100 catheter days), occlusion (0.692/100 catheter days), and dislodgement of needle (0.643/100 catheter days). There were major complications that necessitated removal of catheters, including systemic infections (0.09/100 catheter days) and complete occlusions (0.09/100 catheter days). The system was thought to be safe and convenient in chemotherapy, and permitted full physical activity.
Anti-Bacterial Agents ; Antineoplastic Agents ; Catheters ; Child* ; Drug Therapy ; Humans ; Motor Activity ; Needles ; Prospective Studies*

PURPOSE: Dendritic cells (DCs) are the most potent antigen presenting cells and should be differentiated to mature form to induce primary T cell response. In this study, we intended to generate mature DCs from peripheral blood mononuclear cells (PBMCs), so that develope the basis for immunotherapy using DCs. METHODS: PBMCs were isolated from 25 mL of normal adults' peripheral blood and evenly distributed in 5 wells of a 6-well plate. Nonadherent cells were gently aspirated after 2 hour-incubation under humidified 5% CO2 at 37degrees C. Adherent monocytes were cultured in 3 mL of 10% fetal bovine serum plus RPMI-1640 media containing granulocyte/macrophage-colony stimulating factor (GM-CSF) 200 ng/mL and interleukin (IL)-4 20 ng/mL. To assess the effect of tumor necrosis factor (TNF)-alpha and interferon (IFN)-alpha on DC maturation, either or both were added on day 4 of culture. Cells were harvested on day 4 and 7 to calculate the cell counts, CD83 /HLA-DR cells, and CD86 /HLA-DR cells. RESULTS: On day 4, large amounts of DCs were observed. CD83 /HLA-DR cells and CD86 / HLA-DR cells were 11.6% and 16.6% of total cells counted and yields were 1.3% and 2.0%, respectively. On day 7, DCs were more frequently observed in all instances and purity ranged from 24.0% to 31.0% as a mean value. The final yields of matue DCs were 2.9~3.4% of PBMCs inoculated. Adding TNF-alpha plus IFN-alpha led to the best yield. But, IFN-alpha alone did not increase the mature DCs compared to the control. CONCLUSION: We successfully cultured large quantities of mature DCs from PBMCs using GM-CSF and IL-4. IFN-alpha seems to have a synergistic effect when added with TNF-alpha, but further studies are required to prove the clinical significance.
Antigen-Presenting Cells ; Cell Count ; Dendritic Cells* ; Granulocyte-Macrophage Colony-Stimulating Factor ; HLA-DR Antigens ; Immunotherapy ; Interferon-alpha ; Interferons ; Interleukin-4 ; Interleukins ; Monocytes ; Tumor Necrosis Factor-alpha