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)

Consistent cytogenetic abnormalities have been described in many pediatric solid tumors, including Ewing's sarcoma, Wilms' tumor, and neuroblastoma. Similar analysis of pediatric central nervous system (CNS) tumors has been hampered by technical problems. We report chromosome results from 39 pediatric CNS tumors. Abnormalities of chromosome 17 were noted in 3 of 11 primitive neuroectodermal tumors (including i(17q) in 2 tumors), confirming data observed by other investigators. Cells from 2 of 11 primitive neuroectodermal tumors (PNET) exhibited loss or structural abnormalities involving chromosome 11. Loss or distal deletion of chromosome 7q was noted in cells from two PNETs. Because other investigators have shown loss of heterozygosity on 17p in about one-third of PNET, we propose that chromosome regions 7q and 11 are areas worthy of further study in pediatric PNET. Numerical abnormalities were noted in 6 of 21 astrocytomas. Hyperdiploidy was demonstrated in 1 of 4 pilocytic astrocytomas and pseudopolyploidy was demonstrated in 4 of 13 anaplastic astrocytomas. Structural chromosome abnormalities (translocations, deletions) were noted in 4 of 13 anaplastic astrocytomas. Complex structural anomalies were observed in one craniopharyngioma. A rhabdoid tumor of the brain exhibited multiple complex structural rearrangements but did not exhibit the monosomy 22 observed in some rhabdoid tumors. Hypodiploidy and loss of chromosome 22 were noted in a clinically aggressive meningioma, corroborating observations by other investigators.
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PMID:Cytogenetic analysis of 39 pediatric central nervous system tumors. 131 29

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

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

In normal conditions, neuron-specific enolase (NSE) is histochemically demonstrable only in neurons and cells of the amine precursor uptake and decarboxylation (APUD) system. This has been found not to be true for neoplastic cells. Several types of CNS tumors, including glioblastoma, astrocytoma, oligodendroglioma, ependymoma, medulloblastoma, pineocytoma , meningioma, and choroid plexus papilloma, focally stained positively for NSE. Reactive astrocytes were also frequently positive. In the peripheral nervous system, neuroblastoma, ganglioneuroma, and paraganglioma stained positively for NSE. A number of non-APUD tumors were focally positive. These included schwannoma, carcinoma and fibroadenoma of the breast, renal cell carcinoma, giant cell tumor of the tendon sheath, and chordoma. Caution should be exercised in relying on the immunohistochemical demonstration of NSE as a diagnostic marker in those tumors that do not belong to the APUD cell system. It seems of little value as evidence of differentiation in CNS tumors.
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PMID:Immunohistochemical demonstration of neuron-specific enolase in neoplasms of the CNS and other tissues. 654 18

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

Cerebral neuroblastoma is often difficult to differentiate by clinical criteria from other central nervous system neoplasm. This report is of a young child with cerebral neuroblastoma and elevated nerve growth factor. This case illustrates the need for further studies into the usefulness of nerve growth factor in differentiating cerebral neuroblastoma from other central nervous system tumors.
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PMID:Cerebral neuroblastoma with elevated nerve growth factor. 740 31

The records of all children in Northern Israel under the age of 1 year in whom a malignant solid tumor was diagnosed were analyzed. Between 1973-1990 such tumors were found in 39 boys and 25 girls. The overall annual incidence was 137.1 per million, and the incidence was higher in boys (1.9/1.0), in Jews compared to non-Jews (1.3/1.0), and in Ashkenazic Jews compared to Sephardic Jews (1.2/1.0). Neuroblastoma was the most common (52% of all malignancies), followed by Wilms' tumor (13%), CNS neoplasm (11%), retinoblastoma (8%), soft tissue sarcoma (6%), lymphoma (5%) and all others (6%). The retinoblastomas were all in non-Jews, but Jews had a higher incidence of neuroblastomas. No differences in incidence were observed in other neoplasms.
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PMID:[Malignant solid tumors in the first year of life]. 803 44

As the cure rate for childhood malignancies increases, the number of patients at risk for development of second malignancies also increases. Due to the potentially long remaining life span, long-term follow-up is difficult and patients are often at risk after presumptive cures. Some authors believe that cure rates for second malignancies are similar to cure rates for primary malignancies. We reviewed the records of 162 patients seen at our institution who had developed a second malignancy after treatment for childhood cancer. Presentation, age at diagnosis, tumor histology, extent of tumor, treatment (including radiotherapy with dosage when available, and chemotherapy) plus outcome were recorded. Mean age at diagnosis of the primary malignancy was 10.3 years. The most common primary malignancy was Hodgkin's disease (33) followed by soft tissue sarcoma (28), retinoblastoma (20), bone tumor (17), central nervous system (CNS) tumor (13), leukemia (8), Wilms' tumor (7), non-Hodgkin's lymphoma (6), neuroblastoma (5), thyroid neoplasm (5), and others (20). The average interval between diagnosis of the first and second malignancy was 10.8 years. These second tumors carried a high mortality. Only 56 patients have no evidence of disease. Five patients are known to be alive with disease and 92 patients have expired due to their second malignancy. Disease status in 8 patients is unknown. The most common second malignancy was osteosarcoma (35) followed by soft tissue sarcoma (24), breast cancer (15), leukemia (14), thyroid carcinoma (14), CNS tumors (12), melanoma (8), nonmelanomatous skin cancer (8), lymphoma (5), and others (27).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Forty-year experience with second malignancies after treatment of childhood cancer: analysis of outcome following the development of the second malignancy. 826 99

The causes of most childhood cancer remain elusive; some children clearly have a genetic predisposition, but in the majority the relative contributions of environmental and host factors are not established. One approach to this question is through twin concordance studies, but only the most common malignancy, acute leukemia, has been studied to date, owing to the rarity of other forms of childhood cancer. The aim of the study was to determine the concordance rates for childhood cancer in twins, in order to clarify the importance of constitutional predisposition for a range of tumor types. Twins with cancer were ascertained through three cooperative clinical trials groups, a cancer-twin registry, and a large pediatric hospital. Subjects were sent a postal questionnaire requesting information on cancer concordance and zygosity. Data were obtained on 556 twins with cancer. Three twin pairs, out of 197 twin pairs (76 monozygous, MZ, twin pairs), were concordant for leukemia, giving an MZ case-wise concordance rate (5%) that is substantially lower than previously reported. The case-wise concordance for non-retinoblastoma solid tumors was 2.2%: Two twin pairs were concordant for CNS tumors, one was concordant for neuroblastoma, and two twin pairs were concordant for cancer but not for the type of cancer. The results of the present study, together with previous data from population studies of siblings and offspring, suggest that there is not in general a strong constitutional genetic component for childhood cancers other than retinoblastoma.
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PMID:Concordance for childhood cancer in twins. 860 Mar 32

In spite of the fact that official statistical data show lower cancer morbidity of pediatric population in Russia than in Europe and USA (9.7 against 13.8 and 13.6, respectively), real cancer incidence may be close to mean incidence in Europe and USA as in Moscow it is 14.8 in boys and 12.0 in girls. Lower numbers in some other regions may be attributable to low standards of cancer diagnosis and inadequate case registration. In Moscow where standards of children's hematological service are high the proportion of some malignancies is the following: hemoblastoses-48.5%, CNS tumors-19.8%, neuroblastoma-8.0%, renal tumors-6.5%, sarcomas of soft tissues and bones-2.4 and 4.6%, respectively. The efficacy of anticancer treatment of children in many regions of Russia is poor, but the situation may be changed for the best by introduction of new approaches developed in the Moscow Research Institute of Pediatric Hematology.
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PMID:[Status of the pediatric oncohematologic service in Russia]. 875 50


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