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

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

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

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

PURPOSE: In the course of treatment, patients with hematological or oncological disorders often develop pulmonary complication. The patients who develop a severe pulmonary complication have a poor outlook. The causes of pulmonary complication are either infectious or non-infectious in origin. We have analyzed the etiology and outcome of these patients admitted to the pediatric intensive care unit of Asan Medical Center. METHODS: Medical records of 95 patients on Pediatric oncology service who were admitted to pediatric intensive care unit(PICU) of Asan Medical Center from Jan 1997 to May 2000 were retrospectively reviewed. RESULTS: The mean age of the patients was 8.5 years(2 months-18 years). The underlying malignancies of these 95 patients were as following; acute lymphoblastic leukemia(31 cases), lymphoma (11 cases), acute myeloid leukemia(nine cases), brain tumor(eight cases) and other solid tumors(25 cases). Pulmonary complications included pneumonia, acute respiratory failure, pneumothorax and pleural effusion. The most common cause of pulmonary complication was infection(88%) in etiology. The overall mortality rate was 56.8%. Pulmonary complications in these patients carried high rates of mortality regardless of whether they were immune compromised(76%) or not(69%). Even without pulmonary complications, the hematological or oncological patients admitted to PICU had high mortality rates of 43%. CONCLUSION: Pulmonary complications are frequent finding in the hematological or oncological patients admitted to Intensive Care Unit. The main etiology of these pulmonary complications was infection, which carried a high mortality rate regardless of their immune status at the time when they were admitted to PICU
Brain ; Child* ; Chungcheongnam-do ; Humans ; Intensive Care Units* ; Critical Care* ; Lymphoma ; Medical Records ; Mortality ; Pleural Effusion ; Pneumonia ; Pneumothorax ; Prognosis* ; Respiratory Insufficiency ; Retrospective Studies

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

PURPOSE: The frequency and clinical characteristics of primary childhood hepatic tumors diagnosed in a single institution were reviewed. Method: From January 1991 to June 2000, 33 patients were diagnosed with primary hepatic tumor. Retrospective analysis of the medical records of the various primary hepatic tumors was performed. RESULTS: Among the total of 33 patients with primary hepatic tumor, twenty five (76%) were malignant and eight (24%) were benign. The mean age of patients with hepatoblastoma (HB) was 32 months (12 of 16 patients were less than 3 years), 12.8 years in hepatocellular carcinoma (HCC) (all of them were older than 11 years), 2 months in hemangioendothelioma (HE), and 3 months in hamartoma. The ratio of male to female was 1:1 in HB, and 4:1 in HCC. The presence of hepatitis B infection was detected in 80% (4/5) of HCC and in 13% (2/16) of HB. The common presenting symptoms at diagnosis were the palpable mass, abdominal pain and jaundice. Many tumors were found incidentally without any presenting symptoms. Of 33 patients, 22 had involvement at right lobe. HCC had more necrosis and hemorrhage within the tumor than HB on imaging study. HE had a distinct feature of contrast enhancement increasing from the peripheral portion of the mass to central portion. Hamartoma showed a feature with multiple cysts and septums within the mass. To confirm the diagnosis, a tissue biopsy was done in 23 patients (70%). A significantly elevated level of serum alpha-fetoprotein (AFP) is found in 88% (mean 386,000 ng/mL) of HB cases and 100% (mean 369,000 ng/mL) of HCC. The mean AFP level in HB patients was 391,000 ng/mL at diagnosis, 52 ng/mL after the preoperative chemotherapy, 8.3 ng/mL at postoperative follow-up, and 2.8 ng/mL at completion of chemotherapy. In HCC, the AFP level decreased temporarily after chemotherapy or chemo- embolization, but increasesd at recurrence in most case. Patients resectable at the time of diagnosis or after preoperative chemotherapy were 12 (75%) in HB, and 10 of them are were alive with median the follow-up of 34 months (5 mo.~8 yrs 7 mo.). All the HCC patients were unresectable or had metastatic lesion at diagnosis, and all of them were assumed dead following discharge. Conclusions: Among the primary hepatic tumors in children, malignant tumors are more frequent than benign. Age of onset, AFP level and imaging studies all play an important role in diagnosis, and biopsy is usually confirmative. HCC has an extremely poor prognosis in childhood. HB patients with complete surgical resection enjoy a relatively high long term survival.
Abdominal Pain ; Age of Onset ; alpha-Fetoproteins ; Biopsy ; Carcinoma, Hepatocellular ; Child* ; Diagnosis ; Drug Therapy ; Female ; Follow-Up Studies ; Hamartoma ; Hemangioendothelioma ; Hemorrhage ; Hepatitis B ; Hepatoblastoma ; Humans ; Jaundice ; Male ; Medical Records ; Necrosis ; Prognosis ; Recurrence ; Retrospective Studies