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)

Histocompatible bone marrow transplantation (BMT) is the treatment of choice for pediatric patients with second remission acute lymphoblastic leukemia or acute myelogenous leukemia (AML) and has been successfully used to treat patients with first remission AML and stable-phase chronic myelogenous leukemia. The principle causes of transplantation failure are recurrent leukemia and therapeutic toxicities, including idiopathic interstitial pneumonitis and graft versus host disease (GVHD). The likelihood of leukemic relapse is related primarily to the remission status of the patient; patients in first remission have a lower relapse rate than patients in second remission, and the relapse rate of both is less than that of patients in relapse. Interstitial pneumonitis is due, in part, to the total body irradiation (TBI) that is used to cytoreduce the patients. TBI administration has been modified to reduce its toxicity. Acute and chronic GVHD are due to the immuno-aggression of donor T-lymphocytes against recipient non-HLA antigens. The in vitro removal of the T-lymphocytes from the donor bone marrow inoculum reduces the incidence of acute and chronic GVHD but may have the adverse effect of reducing hematopoietic engraftment and increasing leukemic relapse since the graft versus leukemia effect may be eliminated. The expanding role of BMT includes its use in the treatment of nonleukemic neoplasms (neuroblastoma, solid tumors) and the use of histoincompatible BMT for eligible patients without histocompatible donors.
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PMID:Current status of bone marrow transplantation in pediatric oncology. 352 38

Current therapy for children with cancer includes a variety of invasive procedures many of which require repeated venous access over a considerable period of time. Such procedures are poorly tolerated by children and by their veins. Recently it has become possible to undertake the majority of such procedures by means of permanent indwelling silastic catheters improving the quality of life of the children and their parents and increasing the scope of therapeutic intervention. In the period July '83 - August '84 we have used 46 of these catheters in 45 children with malignant disease, 12 with acute myeloid leukaemia, 12 with neuroblastoma, 7 with B cell leukaemia-lymphoma, 6 with rhabdomyosarcomas, 2 with Ewing's Sarcoma, 2 with Wilms' tumor and 1 case each of Hodgkin's disease, teratocarcinoma, osteosarcoma and juvenile chronic myeloid leukaemia. The children's ages ranged from 2 months to 14 years; 22 were male and 23 female. The catheters were inserted under general anaesthesia (duration 20-40 minutes) usually without difficulty, except for a single patient in whom no suitable vein could be found. No complications connected with the placement of the catheter were observed. Subsequent management of the catheter was initially complicated and time-consuming, but was subsequently simplified so that acceptance by parents, children and nursing staff was eventually excellent. The duration of use of 46 catheters ranges from 7 to 350+ days; 24 catheters are presently in use at 30-350+ days from insertion. Eight children died as a result of disease progression and two of sepsis with the catheter in place.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Advantages of a permanent venous access in children treated for cancer. Preliminary results]. 383 38

At least 22 members of a large kindred have a bleeding tendency resulting from an autosomal dominant disorder of platelet production and function. Phenotypic manifestations include mild to moderate thrombocytopenia, bleeding time prolongation, and abnormal platelet aggregation. Platelet survival time is normal. The platelet disorder in this family appears to differ from known hereditary thrombocytopenic or thrombocytopathic syndromes and may represent a new genetic disease. Six family members reportedly developed hematologic neoplasms: acute monocytic leukemia nine years after treatment for congenital neuroblastoma; lymphosarcoma at age 10 years; myeloid leukemia at age 23 years; acute myelocytic leukemia at age 62 years; leukemia of unknown type at age 48 years; and lymphocytic lymphoma at age 52 years.
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PMID:Studies of a familial platelet disorder. 385 65

The proportion of malignancies in children differs from that in adults: Leukemias and malignant lymphomas predominate with a total of 50%, followed by tumors of the nervous system, of the kidneys, and of connective and supportive tissue. Most of these diseases respond well to cytostatic therapy. Therefore chemotherapy occupies a major role in the curative concepts for nearly all childhood malignancies. Its objective is the destruction of micrometastases as well as the reduction of primary tumor mass in inoperable cases, and it often helps to limit the extent of radical surgery. Radiotherapy, too, can be reduced under the influence of cytostatic therapy. In nearly all childhood cancers, prognosis has improved substantially over the past 10 to 15 years. Today, our aim is not the mere limited survival, but a definitive cure. Modern strategies have raised the cure rates of Hodgkin's disease to 90%, of Wilms' tumor, acute lymphoblastic leukemia and non- Hodgkin lymphomas to 70-75%, of soft tissue sarcomas and osteosarcomas to about 50%, and of acute myelogenous leukemia, neuroblastoma and medulloblastoma to 30-35%. Centralized management of childhood cancers in specially staffed hospitals is mandatory on account of their relative low frequency, the risks of chemotherapy, and the high staff workload.
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PMID:What's new in pediatric oncology? Epidemiology, treatment principles and prognosis in childhood malignancies. 388 97

We have studied the incidence pattern of childhood cancers in Korea. Although the incidence of many tumors in Korea is similar to that in other countries, the incidence of acute myelogenous leukemia, non-Hodgkin's lymphoma and hepatoma is greater in Korean children. Yonsei Cancer Center commenced a study of multi-modality treatment of childhood cancers in July 1974. The most striking improvement of survival rate was seen in patients with acute lymphocytic leukemia (50% at 5 years), Wilms' tumor (65% at 5 years), neuroblastoma (45% at 2 years), osteogenic sarcoma (55% at 2 years) and malignant histiocytosis (20% at 5 years). This study is an attempt to create a basic framework providing the best possible treatment of childhood cancer in Korea. The data obtained in Korea are briefly compared with those in Japan and the United States.
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PMID:The present status of childhood cancer therapy in Korea. 609 45

A variety of solid and hematological human tumors and normal human bone marrow specimens were assayed for colony formation in a short-term soft-agar culture system. The effect of human fibroblast, lymphoid, and myeloid interferons on inhibition of colony formation was assessed. The effect of interferon on colony formation formed a continuum from complete inhibition to stimulation of growth. Of 40 evaluable tumor specimens, 18 showed at least a 70% inhibition of colony formation in the presence of interferon, at concentrations of 1000 units/ml or less. Four specimens (acute myelogenous leukemia, osteogenic sarcoma, neuroblastoma, ovarian carcinoma) showed at least 3-fold stimulation of colony formation with interferon. Two of 12 normal bone marrow specimens grown with colony-stimulating, factor-conditioned media showed greater than 70% colony inhibition with interferon. A dose-response relationship was seen in all tumor specimens tested. While fibroblast interferon was the most active in this system, all interferons showed the same magnitude and direction of activity. Continuous exposure and 1-hr incubation of tumor cells with interferon were identical in terms of colony inhibition. These data support the ability of this assay system to select tumors responsive in vitro to interferon, suggest the optimal species and concentration for inhibition or stimulation of growth, and support a direct role of interferon in the regulation of cell growth independent of other immunoregulatory actions of interferon. Such information may prove useful for predicting response in vivo to interferon in Phase II trials.
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PMID:Effect of fibroblast, lymphoid, and myeloid interferons on human tumor colony formation in vitro. 616 Sep 5

Pretreatment serum samples obtained at diagnosis from 89 children with various pediatric malignancies were examined for circulating immune complexes (CIC) using the [125I]Clq binding assay. The study population consisted of 35 children with acute lymphocytic leukemia (ALL), 22 children with acute non-lymphocytic leukemia (ALL), 24 with neuroblastoma (NB), and eight with osteosarcoma (OS). Concomitant quantitation of immunoglobulins was performed in 55 patients, revealing normal values for age. Increased levels of CIC at diagnosis were found in 9%, 22%, 42%, and 50% of children with ALL, AML, NB, and OS, respectively. Except for a higher proportion of CIC-positive patients observed in stage IV NB (nine of 17) compared to stage I-III NB (one of seven), no correlation was observed between initial CIC level and presenting clinical features, response to treatment, prognosis, or presence of infection. Longitudinal sampling of six NB and two OS patients did not reveal a clear relationship between disease activity and quantity of CIC. For the pediatric malignancies studied, these data demonstrate minimal value in quantitating CIC as a means of assessing disease activity or predicting response to treatment and are in contrast to the apparently adverse effect of elevated pretreatment CIC on response to therapy and survival observed in adults with ALL, AML, and OS.
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PMID:Circulating immune complexes in childhood malignancies: a Pediatric Oncology Group study. 658 17

The oncogene of the HL-60 human promyelocytic leukemia cell line has been passed serially through NIH/3T3 mouse fibroblasts. Oncogene-specific probes prepared from the resulting tertiary transfectants by molecular cloning have been used to show that loss of the transfected oncogene from NIH/3T3 cells correlates with reversion to nontransformed morphology. Analysis of cells transfected by the oncogenes of other tumors and tumor cell lines indicates that the transforming gene of the HL-60 leukemia cell line is closely related to oncogenes of a Burkitt's lymphoma, an acute myelogenous leukemia, an adenocarcinoma of the colon, a neuroblastoma, and two sarcomas. This oncogene is distantly related to the viral oncogenes of Kirsten and Harvey sarcoma viruses. It has been termed N-ras. The active N-ras oncogene coexists with altered versions of the myc oncogene in the HL-60 and AW Ramos human tumors. This suggests a multistep mechanism involving both ras and myc genes in the creation of these tumors.
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PMID:The HL-60 transforming sequence: a ras oncogene coexisting with altered myc genes in hematopoietic tumors. 668 94

A potential application of the human tumor stem cell colony assay is to guide Phase II clinical investigations by identifying classes of tumors (or individual patients) which are sensitive in vitro to a new antitumor compound. We have tested human tumor stem cells from 140 tumor biopsies representing 20 different tumor types for chemosensitivity to the Phase II drug 4'-(9-acridinylamino)methanesulfon-m-anisidide. In vitro sensitivity was defined as a reduction in the number of tumor colony-forming cells to 30% of the control or less after a 1-hr exposure to one-tenth of the pharmacologically achievable plasma concentration of 4'-(9-acridinylamino)methanesulfon-m-anisidide. In vitro sensitivity was found in 29 cases: non-Hodgkin's lymphoma (2 of 2); cervical carcinoma (1 of 1); sarcoma (3 of 6); neuroblastoma (1 of 2); acute myelogenous leukemia (6 of 16); chronic myelogenous leukemia (1 of 3); melanoma (8 of 34); uterine carcinoma (1 of 5); lung carcinoma (1 of 9); ovarian carcinoma (4 of 36); and breast carcinoma (1 of 11). Prospective in vitro-in vivo correlations in eight patients with various tumor types showed that three of three patients sensitive in vitro to 4'-(9-acridinylamino)methanesulfon-m-anisidide responded in vivo, while five of five patients resistant in vitro had no clinical response. The results provide support for further evaluation of the utility of the human tumor stem cell colony assay for targeting Phase II clinical trials.
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PMID:In vitro chemosensitivities of human tumor stem cells to the Phase II drug 4'-(9-acridinylamino)methanesulfon-m-anisidide and prospective in vivo correlations. 689 12

During six-month period, 102 consecutive episodes of fever in 68 children (ranging from 1 month to 14 years of age) with malignant diseases were prospectively evaluated. Sixty-five had acute lymphoblastic leukemia, nine had acute myeloblastic leukemia, nine had malignant lymphoma (four Hodgkin and five non-Hodgkin), five had chronic myeloid leukemia, four had rhabdomyosarcoma, three had CNS tumors, two had neuroblastoma, one had Wilms, and four had other malignant tumors. Forty cases (39.2%) showed severe neutropenia (500 neutrophil/m3) during the episode. S. aureus, E. coli, and S. pyogenes were in 53% of the 75 microbiologic isolates. Twenty-two percent of the viral studies were positive. Mycologic studies were all negative, except one case with C. Albicans. Pneumonia (33 cases), cellulitis (15 cases), pharyngitis (12 cases), and varicella (11 cases) were the most common final diagnosis. Seventy-one percent of the episodes were etiologically documented (by bacterial isolate, characteristic serology, and/or typical clinic picture); 19% of the febrile episodes were probable infections, and 10% were fever of uncertain cause. Ninety percent of the cases responded well to therapy, and mortality of this series was 7%. Gentamicin, Carbenicillin, and Methicilin were the more common antibiotics employed. We conclude that in our population 1) infection is a frequent cause of morbidity in children with malignant diseases; 2) the most common cause of the febrile episodes is bacterial infection; 3) S. aureus, E. coli and S. pyrogenes are the most frequent bacterial isolates, and P. aeruginosa is infrequent; 4)viral infections are relatively frequent in this group of children; and 5) with adequate management, the mortality is low.
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PMID:Infections in children with malignant disease in Argentina. 722 35


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