1.Childhood Brain Tumors.
Journal of the Korean Pediatric Society 2002;45(9):1055-1058
No abstract available.
Brain Neoplasms*
;
Brain*
2.Time to establish multidisciplinary childhood cancer survivorship programs in Korea.
Korean Journal of Hematology 2010;45(2):84-87
Improved treatment strategies and better supportive care have resulted in increased survival rates for childhood cancers. However, most of the survivors may have complex, long-term health issues. In 2004, Childhood Cancer Survivorship Study of the United States confirmed that both survivors and the medical community need to be educated about the late effects of childhood cancer treatment. Korea, with an estimated number of childhood cancer survivors of 20,000 to 25,000, faces similar challenges that the United States had experienced earlier. Despite of the tight budgetary situation on the part of government and hospitals, nationwide cohort study for Korean childhood cancer survivors was proposed and the versions of instruments to measure the quality of life of childhood cancer survivors were already validated. Nationwide registry of long-term survivors as well as the multi-center study is to be developed not only for the care of the survivors but also to raise the patient's awareness of the importance of regular follow-up. In addition to education of primary care providers regarding the survivorship, the need for special education program in the school system is emphasized.
Cohort Studies
;
Education, Special
;
Follow-Up Studies
;
Humans
;
Korea
;
Primary Health Care
;
Quality of Life
;
Survival Rate
;
Survivors
;
United States
3.Atypical Teratoid/Rhabdoid Tumor in Central Nervous System: Report of 2 Cases.
Jae Myung KIM ; Young Shin RA ; Thad T GHIM ; Shin Kwang KHANG
Journal of Korean Neurosurgical Society 2002;32(6):599-602
We report two cases of atypical teratoid/rhabdoid tumor(AT/RT) in the central nervous system. Primary central nervous system AT/RT is an extremely rare malignant tumor affecting infants and young children. These tumors have been diagnosed previously as primitive neuroectodermal tumors(PNETs) because AT/RT contains fields indistinguishable from classic PNETs. Separation of these two tumor types is crucial because the prognosis for AT/RT is poor even when treatment includes surgery with or without radiation therapy and/or chemotherapy. Clinical, radiological and histopathological features of AT/RT are presented.
Central Nervous System*
;
Child
;
Drug Therapy
;
Humans
;
Infant
;
Neural Plate
;
Neuroectodermal Tumors, Primitive
;
Prognosis
4.A Case of Kaposiform Hemangio-Lymphangiomatosis.
Jun Eun PARK ; Jong Jin SEO ; Hyung Nam MOON ; Thad T GHIM
Korean Journal of Pediatric Hematology-Oncology 2000;7(2):287-292
Kaposiform hemangio-lymphangiomatosis is an extremely rare and locally aggressive vascular neoplasm. This neoplasm histologically shows dilated vascular spaces lined by flat endothelium-like cells and areas of spindle cells forming slit-like vascular spaces similar to those described in Kaposi's sarcoma. We report a case of this neoplasm which originated from the mediastinum infiltrating other adjacent soft tissues such as thymus and pulmonary interstitium without the evidence of distant metastasis. In spite of interferon-alpha2b therapy and excision of this neoplasm, the patient expired due to respiratory failure caused by aggravating interstitial pneumonia and progression of the neoplasm.
Hemangioma
;
Humans
;
Lung Diseases, Interstitial
;
Lymphangioma
;
Mediastinum
;
Neoplasm Metastasis
;
Respiratory Insufficiency
;
Sarcoma, Kaposi
;
Thymus Gland
;
Vascular Neoplasms
5.KSPNO Protocol for Glioma.
Byung Kyu CHO ; Hye Lim JUNG ; Thad T GHIM ; Il Han KIM ; Yong Kil HONG ; Young Shin RA ; Mee Jeong LEE
Korean Journal of Pediatric Hematology-Oncology 2005;12(2):244-285
No abstract available.
Glioma*
6.Normalization of Red Cell Enolase Level Following Allogeneic Bone Marrow Transplantation in a Child with Diamond-Blackfan Anemia.
Jeong A PARK ; Yeon Jung LIM ; Hyeon Jin PARK ; Sun Young KONG ; Byung Kiu PARK ; Thad T GHIM
Journal of Korean Medical Science 2010;25(4):626-629
We describe a girl with Diamond-Blackfan anemia with accompanying red cell enolase deficiency. At the age of 9 yr old, the patient received allogeneic bone marrow transplantation from her HLA-identical sister who had normal red cell enolase activity. While the post transplant DNA analysis with short tandem repeat has continuously demonstrated a stable mixed chimerism on follow-up, the patient remains transfusion independent and continues to show a steady increase in red cell enolase activity for over two and a half years following bone marrow transplantation.
*Anemia, Diamond-Blackfan/blood/enzymology/surgery
;
Bone Marrow Cells/cytology/metabolism
;
*Bone Marrow Transplantation
;
Child
;
Child, Preschool
;
Erythrocytes/*enzymology
;
Female
;
Humans
;
Infant
;
Phosphopyruvate Hydratase/genetics/*metabolism
;
*Transplantation, Homologous
7.A Case of Posttransplant Lymphoproliferative Disease Following Renal Transplantation in a Child.
Won Kyoung JHANG ; Hyewon HAHN ; Mee Jeung LEE ; Young Seo PARK ; Thad T GHIM
Journal of the Korean Society of Pediatric Nephrology 2003;7(2):245-252
Posttransplant lymphoproliferative disease(PTLD) has emerged as a potential life-threatening complication of immunosuppressive therapy after organ transplantation. The occurrence of PTLD is usually associated with an Epstein-Barr virus(EBV) infection in patients who are treated by aggressive immunosuppressive therapy. PTLD is represented by diverse manifestations ranging from reactive lymphoid hyperplasia to high grade malignant lymphoma. This is a case report of a late PTLD in a child. The patient is a 14-year-old girl, who presented as malignant lymphoma 44 months after successful renal transplantation. There was no evidence of EBV infection. On bone marrow study, many neoplastic lymphoid cells were detected. Aggressive chemotherapy for PTLD had resulted in clinical remission. However the patient expired from uncontrolled sepsis and septic shock after 77 days.
Adolescent
;
Bone Marrow
;
Child*
;
Drug Therapy
;
Epstein-Barr Virus Infections
;
Female
;
Herpesvirus 4, Human
;
Humans
;
Kidney Transplantation*
;
Lymphocytes
;
Lymphoma
;
Organ Transplantation
;
Pseudolymphoma
;
Sepsis
;
Shock, Septic
;
Transplants
8.Treatment Outcome of Childhood B-cell Lymphoma and L3 Acute Lymphoblastic Leukemia from a Single Institution.
Yeon Jung LIM ; Yoon Jung KIM ; Joon Sup SONG ; Mi Jung LEE ; Jong Jin SEO ; Hyung Nam MOON ; Thad T GHIM
Korean Journal of Pediatric Hematology-Oncology 2005;12(1):28-39
PURPOSE: High survival rate can be obtained in B-cell lymphoma (Burkitt's lymphoma, diffuse large B-cell lymphoma) and L3 acute lymphoblastic leukemia (ALL) with multiagent chemotherapy. Objectives of this study were to evaluate the treatment outcomes of B-cell lymphoma and L3 ALL diagnosed at the Department of Pediatrics, Asan Medical Center. METHODS: The medical records of 32 children who were diagnosed with Burkitt's lymphoma, diffuse large B-cell lymphoma and L3 ALL from March 1992 to July 2004 at Asan Medical Center were reviewed retrospectively. The 5 year event free survival (EFS) according to the diagnosis, age, risk group and lactic dehydrogenase (LDH) level were analyzed. RESULTS: There were 23 boys and 9 girls. Age ranged from 9 months to 14.4 years old with a median of 7.1 years. Fourteen patients had L3 ALL, 11 had Burkitt's lymphoma and 7 had diffuse large B-cell lymphoma. Five patients (15.6%) had CNS involvement and 5 with B-cell lymphoma (27.8%, 5/18) had BM involvement. All patients who received appropriate chemotherapy achieved a complete remission (CR), but 18.8% (6/32) relapsed. Among 6 relapsed patients, 5 achieved CR after reinduction chemotherapy. One who had no response to secondary chemotherapy and 2 with isolated CNS relapse died due to disease progression. The most common treatment-related toxicity was myelosuppression (87.5%) followed by neutropenic fever (81.3%). Median follow up is 25 months (3 months to 74 months). Four patients who achieved CR after proper induction therapy (4/32, 12.5%) died, 3 due to relapse and 1 due to toxicity-related complication (neutropenia and sepsis). The 5 year EFS for all patients was 77.5+/-7.5% and the 5 year overall survival was 84.6+/-7.3%. The 5 year EFS of B-cell lymphoma compared with that of L3, ALL was 94.4+/-5.4% versus 55.1+/-13.9% (P=0.012) and 5 year overall survival of relapsed patients was 50.0+/-13.9%. CNS disease at diagnosis, age, LDH had no significant influence on EFS. CONCLUSION: High survival rate of childhood B-cell lymphomas and L3 ALL was obtained with recent intensive multiagent chemotherapy and about 50% of relapsed patients were salvaged with reinduction. High incidence of the treatment-related toxicity such as myelosuppression, neutropenic fever and TLS was observed, but the treatment-related mortality was very low with recent supportive therapies. Survival rate was improved with prompt and appropriate management for the treatment-related toxicity of the intensive chemotherapy.
B-Lymphocytes*
;
Burkitt Lymphoma
;
Central Nervous System Diseases
;
Child
;
Chungcheongnam-do
;
Diagnosis
;
Disease Progression
;
Disease-Free Survival
;
Drug Therapy
;
Female
;
Fever
;
Follow-Up Studies
;
Humans
;
Incidence
;
Lymphoma, B-Cell*
;
Lymphoma, Non-Hodgkin
;
Medical Records
;
Mortality
;
Oxidoreductases
;
Pediatrics
;
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
;
Recurrence
;
Retrospective Studies
;
Survival Rate
;
Treatment Outcome*
9.The Role of Adjunctive Therapy of Optic Pathway Glioma in Children.
Jong Ho JANG ; Young Shin RA ; Jeong Hun KIM ; Jeong Gyo LEE ; Shin Kwang KHANG ; Thad T GHIM
Journal of Korean Neurosurgical Society 2004;35(2):136-141
OBJECTIVE: This study is performed in order to compare the outcomes of surgical management and to define the role of adjunctive therapy in the management of optic pathway glioma in children. METHODS: Sixteen children with optic pathway glioma had been managed in various treatment methods during the last 8 years. The patients aged from 5 months to 14 years. The patients presented with progressive visual loss, increased ICP symptoms, endocrine dysfunction, seizure, and motor weakness. Optic pathway glioma associated with neurofibromatosis was excluded. Tumor involved chiasmatico-hypothalamus(12 patients), optic chiasm(3), and optic nerve(1). The extent of surgical resection were radical(3 patients), subtotal(12), and partial(1). RESULTS: Three patients treated with radical resection showed no evidence of tumor recurrence. Among 12 patients treated with subtotal resection and without adjunctive therapy, 6 patients(50%) developed recurrence in the postoperative period of average 20.5 months. Those patients with recurrence were managed by reoperation(3 patients), irradiation therapy(2), and chemotherapy(1). However three patients with residual tumors after subtotal(2), or partial(1) resection were treated with adjunctive chemotherapy in the postoperative period showed no evidence of recurrence. CONCLUSION: Although radical resection of optic pathway glioma might offer long-term control of tumor, adjunctive chemotherapy could be effective to prevent tumor recurrence in children with subtotally or partially resected optic pathway glioma. More experience is necessary to determine the optimal method of treatment of optic pathway gliomas in children.
Child*
;
Drug Therapy
;
Glioma*
;
Humans
;
Neoplasm, Residual
;
Neurofibromatoses
;
Postoperative Period
;
Radiotherapy
;
Recurrence
;
Seizures
10.High Dose Chemotherapy with Autologous Peripheral Blood Stem Cell Transplantation in Patients with Medulloblastoma/Primitive Neuroectodermal Tumor.
Ki Woong SUNG ; Keon Hee YOO ; Hong Hoe KOO ; Do Hoon LIM ; Hyung Jin SHIN ; Yoon Jeong KIM ; Seung Do AHN ; Young Shin RA ; Thad T GHIM
Korean Journal of Pediatric Hematology-Oncology 2001;8(2):264-272
PURPOSE: To improve survival and/or to avoid radiotherapy, high dose chemotherapy (HDCT) with autologous peripheral blood stem cell transplantation (PBSCT) was given to patients with recurrent or high risk medulloblastoma (MB)/primitive neuroectodermal tumor (PNET) as well as patients younger than 3 years of age. METHODS: Six patients (3 recurrent, 1 high risk, 2 younger than 3 years; 5 MBs and 1 PNET) received single or double HDCT and PBSCT with or without immunotherapy using interleukin-2. Chemotherapeutic regimen in the first HDCT included cyclophosphamide (1,500 mg/m2/ day for 4 days) and melphalan (60 mg/m2/day for 3 days). Chemotherapeutic regimen in the second HDCT included carboplatin (400 mg/m2/day for 3 days), thiotepa (250 mg/ m2/day for 3 days), and etoposide (200 mg/m2/day for 3 days). RESULTS: Nine HDCTs were applied in 6 patients. Three double HDCTs were rescued with peripheral blood stem cells collected during single round leukapheresis. Rapid hematologic recovery occurred in 4 patients. Engraft failure occurred in 1 patient and delayed granulocyte recovery and platelet engraft failure occurred in 1 patient. Three patients who had minimal disease before HDCT had event free survival for 7~18 months after HDCT. Tumor relapsed 8 and 12 months after single HDCT in 2 patients among 3 patients with recurrent MB/PNET. One patient with recurrent MB died due to engraft failure and sepsis. CONCLUSION: HDCT with autologous PBSCT is expected to improve survival of patients with poor prognosis MB/PNET including younger patients less than 3 years. Subsequent trials with larger number of patients and long-term follow-up are needed.
Blood Platelets
;
Carboplatin
;
Cyclophosphamide
;
Disease-Free Survival
;
Drug Therapy*
;
Etoposide
;
Follow-Up Studies
;
Granulocytes
;
Humans
;
Immunotherapy
;
Interleukin-2
;
Leukapheresis
;
Medulloblastoma
;
Melphalan
;
Neural Plate*
;
Neuroectodermal Tumors*
;
Neuroectodermal Tumors, Primitive
;
Peripheral Blood Stem Cell Transplantation*
;
Prognosis
;
Radiotherapy
;
Sepsis
;
Stem Cells
;
Thiotepa