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.
Down Syndrome* ; Leukemia*

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

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

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*

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

No abstract available.
Brain Neoplasms* ; Brain* ; Drug Therapy ; Medulloblastoma ; Neoplasm Metastasis* ; Neuroectodermal Tumors, Primitive

PURPOSE: We performed laparoendoscopic single-site (LESS) partial nephrectomy in a porcine model with the objectives of overcoming the technical challenges of this surgery and exploring its feasibility. MATERIALS AND METHODS: Six partial nephrectomies were performed on a pig aged 5 months, three cases on each kidney, by four surgeons: two urologists with much experience in laparoscopic surgery (E1, E2) and two less-experienced urologists (B1, B2). While under general anesthesia, the swine was placed in a lateral flank position. Umbilical placement of an Octoport was done with a 2.5 cm incision. After dissection of the renal hilum and Gerota's fascia, a bulldog clamp was applied on the renal artery and parenchymal resection was done. Renorrhaphy was then performed with interrupted sutures with the use of a sliding knot technique. RESULTS: All six partial nephrectomies were performed successfully after repair of the vascular and collecting system at the resection margin and renorrhaphy without the need to introduce any additional ports. There were no noticeable intra- or postoperative complications. The mean warm ischemic time was 42 minutes (range, 30-60 minutes). The shortest warm ischemic time, 30 minutes, was achieved by using the early unclamping technique during renorrhaphy. The longest warm ischemic time was 60 minutes. The average number of renorrhaphy stitches was 3.2 (range, 2-5). CONCLUSIONS: LESS partial nephrectomy was successfully performed in a porcine model but resulted in a longer ischemic time than that of conventional laparoscopic surgeries. Therefore, further laboratory disciplinary efforts are needed to decrease the warm ischemic time and to improve this surgical technique.
Aged ; Anesthesia, General ; Fascia ; Humans ; Kidney ; Kidney Neoplasms ; Laparoscopy ; Nephrectomy ; Postoperative Complications ; Renal Artery ; Sutures ; Swine ; Warm Ischemia