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)

Wiedemann-Beckwith syndrome (WBS) may be associated with abdominal tumors, including Wilms tumor, adrenocortical carcinoma, hepatoblastoma, gonadoblastoma, rhabdomyosarcoma, and neuroblastoma. We report on a newborn infant with WBS and a congenital teratoma of the stomach. This is the sole report of any teratoma being associated with WBS and also the first report of a tumor present at birth and visible prenatally in WBS. At birth this infant boy had the diagnostic findings of WBS with macroglossia, ear lobule creases and pits, nevus flammeus, and omphalocele, and an abdominal mass. Abnormalities were detected prenatally when ultrasound examination showed placental overgrowth, polyhydramnios, omphalocele, and posterior abdominal calcifications. Resection of the mass and partial gastrectomy were performed at age 10 days; histologic study showed an immature grade-II teratoma containing a mixture of mature and immature tissues from all germ layers. Results of cytogenetic studies of blood and teratoma were normal (46,XY). This congenital gastric teratoma in a newborn boy with classical WBS may represent either a tumor or an included twin. We discuss its implications for the association of WBS with neoplasia and monozygotic (MZ) twinning, review various neoplasias associated with WBS, and consider pathogenetic mechanisms.
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PMID:Congenital gastric teratoma in Wiedemann-Beckwith syndrome. 201 33

We have examined expression of the smg p25A (a ras p21-like GTP-binding protein) gene in neural crest-derived tumor cell lines and neuroblastoma tissues. The human neuroblastoma cell lines GOTO, IMR-32, NB-1, and SK-N-SH expressed the 1.6-kilobase smg-25A mRNA. SH-SY5Y and SH-IN, variant cell lines with a neuronal phenotype derived from SK-N-SH, expressed much more smg-25A mRNA than did SH-EP1, a variant line with an epithelium-like phenotype also derived from SK-N-SH. The primitive neuroectodermal tumor cell lines SK-N-MC and KU-SN and the Ewing's sarcoma cell lines RD-ES and SK-ES expressed the smg-25A mRNA to a much smaller extent than did neuroblastoma cell lines. Of 15 human neuroblastoma specimens tested, 13 expressed the smg-25A mRNA to various extents. When the relative ratio of the smg-25A mRNA level to the glyceraldehyde-3-phosphate dehydrogenase mRNA level was compared among neuroblastoma tumor tissues, the value was significantly higher in tumors histologically diagnosed as ganglioneuroblastoma. The smg-25A mRNA was not detected in the tissues of Hodgkin's lymphoma, Wilms' tumor, Ewing's sarcoma, or undifferentiated sarcoma of the liver. These results suggest that the smg-25A mRNA level is closely related to the neuronal differentiation state of tumors derived from the neural crest.
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PMID:Expression of the smg p25A (a ras p21-like GTP-binding protein) gene in human neuroblastoma cell lines and tumor tissues. 212 31

Studies were made on the appearance of second malignant tumors (SMT) in children followed in a pediatric hospital at metropolitan Santiago, Chile, between years 1968 and 1987. A retrospective analysis identified SMT in 7 of 430 patients who survived a childhood cancer (incidence 1.62%). An 8th patient was added, whose first neoplasm was treated in another hospital. The initial diagnosis in the affected children were medulloblastoma, neuroblastoma, Wilm's tumor retinoblastoma, Ewing's sarcoma, Hodgkin's disease and, in two cases, acute lymphocytic leukemias. The age range was 6 months to 11 years. Treatment was done by surgery in 5/8, chemotherapy in 7/8 and radiotherapy in all patients. The latent period between the diagnosis of the first cancer and the diagnosis of the SMT was 3.5 to 12 years (median 8.5 years). Osteosarcomas were the most frequent SMT (5/8). The other SMT were a rhabdomyosarcoma, a non Hodgkin lymphoma and an astrocytoma. The majority of SMT were located in the area of prior radiotherapy (6/8). In the other two cases, one had an osteosarcoma, after a bilateral retinoblastoma, which grew outside the previously treated area, and the last one consisted of a lymphoma which was identified 9 years after an acute lymphocytic leukemia. Only 3/8 SMT patients are alive after 14.21 and 34 months follow up. The other children died between 11 and 20 months after diagnosis of SMT. Notwithstanding these kinds of outcome, benefits of therapy for patients with primary tumors greatly outweight the later risk of cancer induction in a small proportion of them.
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PMID:[Second cancer in pediatric patients]. 213 86

One hundred forty-three patients who received radiation therapy for childhood tumors, and survived to the age of skeletal maturity, were studied by retrospective review of oncology records and roentgenograms. Diagnoses for the patients were the following: Hodgkin's lymphoma (44), Wilms's tumor (30), acute lymphocytic leukemia (26), non-Hodgkin's lymphoma (18), Ewing's sarcoma (nine), rhabdomyosarcoma (six), neuroblastoma (six), and others (four). Age at the follow-up examination averaged 18 years (range, 14-28 years). Average length of follow-up study was 9.9 years (range, two to 18 years). Asymmetry of the chest and ribs was seen in 51 (36%) of these children. Fifty (35%) had scoliosis; 14 had kyphosis. In two children, the scoliosis was treated with a brace, while one developed significant kyphosing scoliosis after laminectomy and had spinal fusion. Twenty-three (16%) patients complained of significant pain at the radiation sites. Twelve of the patients developed leg-length inequality; eight of those were symptomatic. Three patients developed second primary tumors. Currently, the incidence of significant skeletal sequelae is lower and the manifestations are less severe than reported in the years from 1940 to 1970. The reduction in skeletal complications may be attributed to shielding of growth centers, symmetric field selection, decreased total radiation doses, and sequence changes in chemotherapy.
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PMID:Skeletal sequelae of radiation therapy for malignant childhood tumors. 213 23

In recent years there has been a significant improvement in the survival rate of children with malignant solid tumors. With Wilms' tumor, the survival rate has risen to 80%, but a subset of these patients with unfavorable histologies and therefore a higher rate of relapse need a different strategy. For those patients with soft tissue sarcoma, brain tumors, and bone tumors the combination of preoperative chemotherapy, surgery, and radiotherapy followed by maintenance multiagent chemotherapy has resulted in a survival rate of 45% to 70%. In the case of neuroblastoma, a similar aggressive approach has not resulted in an improved survival. A different approach that uses screening of infants by urinary testing for VMA and HVA to detect earlier and potentially less malignant tumors has begun in Japan and North America in the hope that preclinical detection will reduce mortality.
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PMID:Advances and management of solid tumors in children. 215 15

Renal and adrenal anatomy, both normal and abnormal, are well depicted by MRI. Although MRI is not a sensitive modality for detection of renal cell carcinoma, it has clinical utility for evaluation of vascular involvement or direct extension of neoplasm into adjacent organs when CT findings are equivocal. Use of signal characteristics has not been useful in differentiating simple from complex cystic renal masses or among the various causes of medical renal disease and renal transplant failure. Similarly, signal characteristics are not sufficiently reliable for differentiating benign from malignant adrenal masses. MRI is useful, however, for detection and localization of pheochromocytomas. MRI plays a major role in imaging of children with neuroblastoma and Wilms' tumor and may obviate other, often more invasive, examinations in these cases.
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PMID:Magnetic resonance imaging of the kidneys and adrenals. 216 Dec 46

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

DNA, extracted from tumours arising in 29 paediatric patients [14 neuroblastoma, 9 Wilms tumour (nephroblastoma), 6 miscellaneous] was investigated for evidence of N-myc amplification, using pNb-1, a recombinant plasmid containing a 1.0 Kb fragment homologous to the 5' end of the human N-myc gene. Within the neuroblastoma group, 4 patients had 15 or more copies of N-myc which correlated with advanced disease stage, and 3 other patients showed low grade amplification (2-5 copies). Low grade amplification was also observed in one patient with stage III unfavourable histology Wilms tumour, resistant to treatment. N-myc was present at single copy level in all other tumours studied. It is concluded that N-myc activation by amplification confers aggressive properties on a variety of embryonal tumours, rather than being restricted to initiation of neoplasia in tumours of neuroectodermal origin. A greater understanding of the complex interaction of a number of oncogenes involved in neuroblastoma may enable more effective therapeutic strategies to be devised.
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PMID:N-myc oncogene amplification in paediatric tumours. 217 80

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

Ninety nine case of various solid tumors in children were clinically and histologically investigated and the findings were compared with the data obtained by flow-cytometric DNA analysis using paraffin-embedded tumor tissue. Most of the embryonal tumors such as neuroblastoma, nephroblastoma, or medulloblastoma tended to show a diploid DNA stemline (65.2%) in comparison with non-embryonal tumors (45.2%) (p less than 0.025). Aneuploid cases of embryonal tumors in children disclosed histologically rather differentiated appearances, and with a favorable clinical outcome. On the other hand, non-embryonal tumors in children revealed in general an aneuploid DNA stemline and higher rate in high grade malignancy, resembling that of the malignant neoplasms in adult. These prominent features of the flow-cytometric DNA analysis of the embryonal tumors in children suggest that there are some differences in the tumor cytogenetics or histogenesis between the embryonal tumors and the non-embryonal tumors.
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PMID:[Flow cytometric DNA analysis of pediatric solid tumor]. 217 99


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