Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the last 5 years massive chemotherapy and autologous bone marrow transplantation have been increasingly explored in the treatment of pediatric solid tumors, mainly for neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma, Wilms' tumor, germ cell tumors, osteosarcoma, and retinoblastoma. Although the disease course could be changed successfully in most instances, the long-term survival has not yet been improved much over the best available conventional treatments. Despite this, in responding relapsed patients this approach seems promising and may represent the only chance of cure. New and better induction regimens are needed.
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PMID:Autologous bone marrow transplantation in pediatric solid tumors. 216 30

Chromosome abnormalities found in pediatric solid tumors include deletions, translocations, homogeneously staining regions (hsr)/double minutes (dms), and ploidy abnormalities. The discovery of a 13q14 deletion found in lymphocytes of patients with retinoblastoma and developmental delay has led to the cloning of the retinoblastoma gene. Likewise the discovery of an 11p13 deletion in lymphocytes of patients with Wilms' tumor and aniridia has led to the cloning of the Wilms' tumor gene. Chromosome deletions found in tumor cells are considered to play a role on the homologous deletion of cancer suppressor genes. Recently, various translocations have been found mostly in soft tissue sarcomas; i.e. t(11;22) in Ewing's sarcoma, t(2;13) in alveolar rhabdomyosarcoma, t(3;8) in pleomorphic adenoma, t(3;12) in lipoma, t(12;16) in liposarcoma, t(12;14) in leiomyosarcoma, and t(X;18) in synovial sarcoma. These translocations provide important information on the difficult diagnosis of soft tissue sarcomas, and on the selection of chemotherapy protocol. Tumor cells in advanced stage neuroblastomas often show hsr/dms, in which N-myc amplification occurs. While near triploidy was regularly found in early-stage neuroblastomas, near-diploidy or near-tetraploidy was usually found in advanced stage tumors. Among various prognostic factors, N-myc copy numbers and tumor cell ploidies had the largest influence on the prognosis of neuroblastoma patients. Cytogenetic and molecular genetic analyses on tumor cells are becoming increasingly important for the diagnosis of pediatric solid tumors, and the prediction of the patients' prognosis.
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PMID:[Cytogenetics in pediatric solid tumors]. 217 98

Survival rates were analysed for a population-based series of over 15,000 childhood cancers registered in Great Britain during 1971-85. There were highly significant improvements (P less than 0.001 for trend) in survival for many major diagnostic groups. Between 1971-73 and 1983-85 the actuarial 5-year survival rates increased from 37% to 70% for acute lymphoblastic leukaemia, from 4% to 26% for acute non-lymphoblastic leukaemia, from 76% to 88% for Hodgkin's disease, from 22% to 70% for non-Hodgkin's lymphoma, from 61% to 72% for astrocytoma, from 24% to 42% for medulloblastoma, from 15% to 43% for neuroblastoma, from 58% to 79% for Wilms' tumour, from 17% to 54% for osteosarcoma, from 26% to 61% for rhabdomyosarcoma, from 59% to 94% for malignant testicular germ-cell tumours and from 43% to 77% for malignant ovarian germ-cell tumours. These increases in population-based survival rates reflect the substantial advances in treatment of a wide range of childhood cancers since 1970. The two principal diagnostic groups for which there was no evidence of any trend were retinoblastoma, which already had an excellent prognosis with a 5-year survival rate of over 85%, and Ewing's sarcoma, for which the survival rate remained below 45%.
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PMID:Trends in survival for childhood cancer in Britain diagnosed 1971-85. 217 43

Owing to the recent progress of chemotherapy, a concept of surgical intervention to the malignant tumor in childhood needs to be changed. Surgical approach has not always been a sole way to the cure of the disease anymore. I discussed the surgical approach in neuroblastoma, Wilms' tumor, hepatoblastoma, rhabdomyosarcoma and malignant teratoma, stressed especially on how to combine surgery with chemotherapy and on intensiveness of surgery. Since most of the children with malignant tumor can be cured and survive, surgeons have to choose the operative procedure, taking their functional quality into consideration.
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PMID:[Recent surgical approach for pediatric malignant tumor]. 217 75

The neu gene in rat neuro/glioblastoma was found to be activated by a single point mutation in the DNA sequence encoding the transmembrane region of the neu-encoded p185 protein. The human homologue of the rat neu gene, termed c-erbB-2 or HER-2, can also be activated in vitro by a similar mutation in the corresponding region. Although the human neu gene was shown to be amplified/overexpressed in a large portion of human breast and ovarian cancer, no reports indicate that the human neu gene is activated by a point mutation in human tumor. To study the possible point mutation of neu gene in human tumors, we characterized the genomic structure in the transmembrane region of human neu gene, which in turn allowed us to determine DNA sequence in this region directly following DNA amplification by polymerase chain reaction. We analyzed 7 tumor cell lines (2 breast cancer, 1 neuroblastoma, 1 rhabdomyosarcoma, and 3 glioma) and 11 tumor tissue samples (8 breast and 3 ovarian cancers). No mutation was found in the transmembrane region of human neu gene. Our results suggest that unlike the rat neuro/glioblastoma, the single point mutation in the transmembrane region of the human neu gene is a rare event in human tumors. In this study, we developed a technique for direct DNA sequencing of the transmembrane region of the human neu gene. This technique makes it possible to screen a large number of tumor samples.
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PMID:Direct sequencing analysis of transmembrane region of human Neu gene by polymerase chain reaction. 220 83

Carboplatin was administered at 1,000 mg/m2/course in combination with etoposide at 300 mg/m2/course to 23 patients aged 5 months to 16 years. Five patients were affected by neuroblastoma, four by CNS tumors, three by Ewing's sarcoma, two by rhabdomyosarcoma, two by malignant teratoma, two by Wilms' tumor, two by head and neck carcinoma, one by hepatoblastoma, one by synovial sarcoma, and one by Langerhans-cell histiocytosis. Eleven patients were pretreated, seven of them with high-dose cisplatin. The overall response rate was 7/11 (64%) for pretreated and 10/12 (83%) for previously untreated patients. Myelosuppression was the main side effect, with anemia and thrombocytopenia more pronounced than leukopenia. Gastrointestinal toxicity and ototoxicity were very mild; nephrotoxicity and neurotoxicity other than hearing loss were not observed. In children with malignant tumors, the therapeutic activity of carboplatin at high doses, even in combination chemotherapy, deserves further studies.
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PMID:A pilot study of high-dose carboplatin and pulsed etoposide in the treatment of childhood solid tumors. 220 54

Experience with high-dose cytosine arabinoside (HDAC) in pediatric solid tumors is limited. Sixteen children with solid tumors resistant to conventional therapies were registered in a pilot Pediatric Oncology Group (POG) study that required the administration of HDAC at 3 g/m2 every 12 hours for four doses. There were four cases of rhabdomyosarcoma, two cases of fibrosarcoma, four cases of neuroblastoma, and one case each of germ cell tumor, Wilm's tumor, retinoblastoma, hepatocellular carcinoma, Ewing's sarcoma, and Burkitt's lymphoma. All eligible patients had advanced diseases and had previously received extensive chemotherapy. Thirteen patients received one course of HDAC and three patients received two courses of HDAC. Due to prior treatments, patients had less than normal marrow reserves. Short-term toxicity included nausea, vomiting, suppression of hemopoiesis, drug fever, and increased blood urea nitrogen (BUN), creatinine, and liver enzymes. All evaluable patients recovered from their toxicities. There were no drug-related deaths. None of the patients had neurologic problems, including the only patient with prior irradiation to the skull. With the above schedule, HDAC appears to have manageable toxicity.
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PMID:Toxicity of high-dose cytosine arabinoside in the treatment of advanced childhood tumors resistant to conventional therapy. A Pediatric Oncology Group study. 222 60

Five hundred and fifty two bone marrow (BM) specimens (497 aspirates, 55 biopsies) from 518 patients with nonhaematological malignancies were examined to determine the frequency of metastatic deposits. BM involvement was highest in neuroblastoma (9/14), prostate cancer (2/4), retinoblastoma (3/7), Ewing's sarcoma (14/47), rhabdomyosarcoma (5/20) and small cell carcinoma of lung (3/18). BM aspiration smears were adequate in paediatric tumours (neuroblastoma, retinoblastoma, rhabdomyosarcoma) while BM biopsies were most useful in patients with Ewing's sarcoma, prostate cancer and small cell lung cancer. We conclude that BM is an easy investigation in the diagnosis and staging of nonhaematological cancers.
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PMID:Frequency of bone marrow involvement in non-haematological malignancies. 224 93

To develop effective interleukin-2 (IL-2) protocols for pediatric malignancies, it is important to define IL-2 pharmacokinetics in children. In a phase I trial, we studied IL-2 pharmacokinetics in seven children, aged 6-18, five with leukemia, one with neuroblastoma, and one with rhabdomyosarcoma. IL-2 was administered as a 15-min i.v. infusion of either 1 X 10(6) CU/m2/dose or 3 X 10(6) CU/m2/dose (every Monday, Wednesday, and Friday for 3 weeks). IL-2 levels were determined using an IL-2-dependent murine T lymphocyte cell line bioassay. Peak IL-2 levels of 120-426 and 330-740 CU/ml were achieved after the lower and higher doses, respectively. Pediatric IL-2 kinetics resembled data reported for adults, fitting a two-compartment model (least-squares-regression technique), with an alpha half-life of 14.0 +/- 5.6 min (range, 6.3-23.1) and a beta half-life of 51.4 +/- 10.7 min (range, 33.0-66.0). The volume of distribution approximated total extracellular fluid (mean, 0.18 L/kg). Further clinical trials are needed to identify which pediatric malignancies are sensitive to immunotherapy and to establish the optimal treatment regimens.
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PMID:Pharmacokinetics of recombinant interleukin-2 in children with malignancies: a Pediatric Oncology Group study. 225 63

Transcripts for the muscle regulatory gene MyoD1 are expressed during normal skeletal muscle myogenesis and in rhabdomyosarcomas but not in other tissues or in soft-tissue sarcomas. Here we report the distribution of MyoD1 protein, determined by reactivity with anti-MyoD1 polyclonal sera in normal tissues, rhabdomyosarcoma cell lines, and in a variety of pediatric solid tumors. The distribution of MyoD1 protein was highly restricted in normal tissues and was detected only in fetal skeletal muscle and more faintly in adult skeletal muscle. All six human rhabdomyosarcoma cell lines analyzed expressed MyoD1 mRNA transcripts as well as immunoreactive protein. The immunohistochemical expression of MyoD1 protein was then examined in 49 surgical specimens from a variety of pediatric solid tumors. Each of 16 rhabdomyosarcoma specimens was positive for MyoD1, including four that did not express the intermediate filament protein desmin. Two of five specimens originally designated sarcoma type indeterminate (STI) and two of three specimens originally designated extraosseous Ewing's sarcoma (EOE) were positive for MyoD1, suggesting commitment to myogenic differentiation. Three of eight Wilms' tumors, which also expressed desmin and had clearly evident myogenic elements, also were positive for MyoD1. Tumors that failed to express MyoD1 protein included neuroblastoma, primitive neuroectodermal tumor, non-Hodgkins lymphoma, embryonal sarcoma of the liver, malignant fibrous histiocytoma, malignant rhabdoid tumor, and Ewing's sarcoma of the bone. These results indicate that expression of MyoD1 protein is highly restricted in normal human tissues and that expression of this gene product in malignant tissue may be diagnostic for rhabdomyosarcoma. Furthermore MyoD1 staining may be a valuable adjunct in the classification of pediatric soft-tissue sarcomas.
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PMID:Myogenic regulatory protein (MyoD1) expression in childhood solid tumors: diagnostic utility in rhabdomyosarcoma. 226 Jun 21


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