No abstract available.
Brain Neoplasms* ; Brain*

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

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

No abstract available.
Glioma*

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

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

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

PURPOSE: With the use of combined-modality therapy, the survival of the patients with the rhabdomyosarcoma (RMS) has been improved during past 30 years. The reports on the therapeutic outcome of RMS in Korea during past 10 years, however, have been scarce. The authors reviewed the clinical characteristics and treatment outcome of pediatric RMS patients diagnosed in our institution during recent 11 years' period and compared this data with previous reports. METHODS: From January 1990 to December 2000, 29 patients were diagnosed with RMS by histopathology. Retrospective analysis of the medical records of these patients was performed. RESULTS: A male to female ratio was 1.4:1. Age at diagnosis ranged from 8 months to 19 years 5 months and the median age was 8 years 5 months. Head and neck region that included orbit and parameningeal areas was the most common primary site (44.8%) and other common sites were genitourinary tract, extremities and perianal areas. The two major pathologic types of RMS were embryonal (72.4%) and alveolar (17.2%). The patient distribution of Intergroup Rhabdomyosarcoma Study (IRS) clinical group was as follows: Group I 13.8%, Group II 24.1%, Group III 27.6% and Group IV 34.5%. Of the 29 patients, 4 patients were lost to follow up or received no treatment. Among 25 patients treated and followed, 3-year overall survival rate of these patients was 84% and 3-year event-free survival (EFS) was 60%. Three-year EFS of Group I was 100%, Group II 71%, Group III 83% and Group IV 22%. CONCLUSION: The clinical characteristics of RMS in our institution were similar to those reported by IRS. But there were higher proportion of Group IV patients than IRS suggesting the delayed diagnosis of RMS in Korean patients. The treatment outcome was comparable to that of current IRS trials. This results suggest that the treatment outcome of RMS patients in Korea has improved largely owing to the adoption of current IRS protocols.
Delayed Diagnosis ; Diagnosis ; Disease-Free Survival ; Extremities ; Female ; Head ; Humans ; Korea ; Lost to Follow-Up ; Male ; Medical Records ; Neck ; Neoplasm Staging ; Orbit ; Prognosis ; Retrospective Studies ; Rhabdomyosarcoma* ; Survival Rate ; Treatment Outcome

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

PURPOSE: The effect of salvage chemotherapy using mitoxantrone based regimen for refractory AML (RAML) was analyzed. METHODS: Between January 1990 and March 2001, we treated 26 RAML. Ten patients received salvage chemotherapy with mitoxantrone based regimen and 16 patients with regimen devoid of mitoxantrone. RESULTS: Of total 54 AML patients treated during this period, 48 cases were available for analysis. RAML patients were 26 cases (54.1%) and M:F ratio was 2.3:1. FAB M2 and M4 were 8 each, and 7 cases were M7. There were 1 case of M1, M5 and M6 each. Eleven (42.3%) of them showed chromosomal abnormalities with variable karyotypes. Patients on mitoxantrone regimen all achieved complete remission (CR), while mitoxantrone devoid regimen achieved CR in only 56.2%. Allogeneic bone marrow transplantation was done in 5 cases (19.2%) of RAML, who are all alive with the median survival duration of 18 months. In mitoxantrone based regimen, 4 cases developed congestive heart failure and 5 had severe infection due to prolonged myelosuppression. In non-mitoxantrone group, 5 of 7 cases who failed to achieve CR died of sepsis. CONCLUSION: The mortality of RAML patients who are refractory to salvage chemotherapy remains very high. This study suggests that mitoxantrone based chemotherapy is effective in achieving complete remission as well as in prolonging survival in RAML patients.
Bone Marrow Transplantation ; Child* ; Chromosome Aberrations ; Drug Therapy* ; Drug Therapy, Combination ; Heart Failure ; Humans ; Karyotype ; Leukemia, Myeloid, Acute* ; Mitoxantrone* ; Mortality ; Remission Induction* ; Sepsis