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)

Human cDNA clones for NSCL-1 and NSCL-2, two basic domain helix-loop-helix (bHLH) genes expressed predominantly in the developing nervous system, were obtained from a fetal brain cDNA library. The full-length transcripts and the genomic structures were determined. The cDNAs for the two genes encode predicted proteins of similar size (133 and 135 amino acids for NSCL-1 and NSCL-2, respectively) and structure. The carboxyl-terminal 75 amino acids of the two proteins contain the bHLH motif and differ from each other by only three conservative amino acid changes, while the amino-terminal portions are markedly divergent from each other. In addition to the similar protein structure, the genes have a similar genomic organization, suggesting a close evolutionary relationship. The 5'-regulatory regions of the two genes share some features (i.e. potential TATA, CCAAT, and GATA binding sites) but also differ significantly in their G+C content. NSCL-1 is relatively G+C-rich (63%) in the sequences upstream of transcription initiation and has multiple potential binding sites for transcription factors that bind to G+C-rich sequences (e.g. AP-2). NSCL-2 is relatively A+T-rich (63%) in this region and has a potential binding site for AP1. Studies of expression in normal tissues demonstrated expression of NSCL-1 and NSCL-2 in the developing central and peripheral nervous system, most likely in developing neurons. Additional Northern analysis studies in cell lines revealed expression of these genes in some cell lines derived from tumors with neural or neuroendocrine features such as neuroblastoma, PNET, and small cell lung cancer. NSCL-1 is expressed in a larger number of these cell lines. The differences in expression may parallel differences in developmental regulation.
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PMID:A comparative structural characterization of the human NSCL-1 and NSCL-2 genes. Two basic helix-loop-helix genes expressed in the developing nervous system. 132 19

In conclusion, the group of peripheral primitive neuroectodermal tumors has been redefined in recent years on the basis of cytogenetic, molecular genetic and more precisely defined histopathologic characteristics. Although in the past, many tumors has been called Ewing's sarcoma, currently this diagnosis is limited to tumors which cannot be more specifically classified on the basis of their ultrastructural and immunophenotypic characteristics. Most small round cell tumors previously classified as Ewing's sarcoma are now classified as peripheral PNET. The consistent cytogenetic abnormality in Ewing's sarcoma and peripheral PNET and patterns of neurotransmitter enzymes have supported a common neuroectodermal origin. The precise characterization of soft tissue Ewing's sarcoma is further complicated by the several primitive rhabdomyosarcomas that may exhibit a similar light microscopic appearance. The importance of histopathologic distinction among these various round cell tumors of childhood is well recognized. Furthermore, primitive tumors with overlapping neural and mesenchymal features, known as malignant ectomesenchymoma, are now identified more often than previously. Finally, molecular biologic and cytogenetic differences between peripheral PNET and neuroblastoma have confirmed their clinical and biologic differences, in spite of their morphologic similarities. Molecular genetic and flow cytometric evaluation have contributed to the distinction of groups with prognostic significance and offer possibilities for new clinical trials.
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PMID:Peripheral primitive neuroectodermal tumors. Diagnosis, classification, and prognosis. 133 43

Expression of insulin-like growth factor I (IGF-I) mRNA by some tumor cell lines of neuroectodermal origin has been described. To further explore the significance of IGF-I mRNA expression in these tumors, a more extensive analysis was performed. Most (9 of 10) neuroectodermal tumor cell lines with a t(11;22) translocation (primitive neuroectodermal tumor [PNET], Ewing's sarcoma, esthesioneuroblastoma) expressed IGF-I mRNA, whereas 0 of 15 cell lines without the translocation (PNET, neuroblastoma) expressed IGF-I. Furthermore, inasmuch as all neuroblastoma (12 of 12) cell lines examined expressed IGF-II RNA, the pattern of IGF expression could distinguish between these closely related tumors. CHP-100, a PNET cell line with the t(11;22) translocation, was shown to secrete both IGF-I protein and an IGF binding protein, IGFBP-2. This cell line also expressed the type I IGF receptor mRNA, and blockade of this receptor by a monoclonal antibody (alpha IR3) inhibited serum-free growth. These data demonstrate that IGF-I expression is a property of neuroectodermal tumors with a t(11;22) translocation and that interruption of an IGF-I autocrine loop inhibits the growth of these tumor cells.
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PMID:Insulin-like growth factor I expression by tumors of neuroectodermal origin with the t(11;22) chromosomal translocation. A potential autocrine growth factor. 217 8

A survey of in vitro cytotoxic effects of camptothecin in human epitheliod sarcoma, colon, breast and ovarian carcinomas, glioblastoma, and neuroblastoma (PNET) cell lines, was done. We chose the MTT assay to measure survival and observed that 24 h exposures to camptothecin caused consistently greater toxicity than 1 h exposures. The LD50 for camptothecin was in the 12.5-25 ng/ml range. There was a 10-fold range of growth rates measured by OD after 5 days exposure and varied expression of MDR1 in these cell lines--none of which could be correlated with tumor sensitivity to drug. The most sensitive cell lines were colon and glioblastoma, and the most resistance were ovarian, breast and epithelioid sarcoma.
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PMID:Camptothecin cytotoxic effects in vitro: dependency on exposure duration and dose. 757 68

The ability to accurately assess tumor size and orientation to surrounding vital structures is an important consideration during preoperative evaluation. The authors report on nine children with solid tumors (hepatoblastoma [1], neuroblastoma [2], adrenal cortical carcinoma [2], liver adenoma [1], primitive neuroectodermal tumor [PNET] [1], and stage V Wilms' tumor [2]) for whom tumor resectability was questioned because of the tumors' close proximity to major blood vessels (noted through conventional radiographic imaging). The children had scanning with spiral volumetric acquisition computerized tomography, (CT) which obtains images during continuous rotation of the x-ray source while the patient moves at a constant velocity through the gantry. This technique is rapid (18 to 30 seconds), and is similar with respect to radiation exposure; little or no sedation is required, and the contrast dose is lower than that of conventional CT. Three-dimensional reconstruction of spiral CT imaging provided useful information that allowed successful resection in all nine cases. The authors suggest that spiral CT may become an important imaging modality in the preoperative evaluation of pediatric solid tumors and that further evaluation of this new methodology is warranted.
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PMID:The role of spiral (helical) computerized tomography with three-dimensional reconstruction in pediatric solid tumors. 773 57

Immature teratoma occurs occasionally in the brain of children and contains a large amount of immature neuronal tissue. These primitive neuronal components are a good target for studying the morphology of primitive neuroectodermal tumors, including neuroblastoma, ependymoblastoma, medulloepithelioma and so on. Primitive neural tubes are immunohistochemically and ultrastructurally studied in two cases of primary immature teratoma of the child brains, compared to true rosettes in a case of neuroblastoma, primitive neural tube in the fetal rat brain (9 to 13 days of gestational age). The study also extends to the pathology of PNET. Ultrastructurally the primitive neural tube like structures in two teratomas were virtually identical those of developing fetal rat brains and true rosettes in a neuroblastoma. However, these tubular structures are different from each other in immunohistochemistry. These differences are considered to reflect the different developmental lineage of the tumor cells, that is, neuroblastoma produces only neuroblastic cells, and primitive neural tubes in teratoma both neuroblastic and glioblastic cells. Rejuvenation of neuroblastoma cells seems to render a VIM-positivity of the tumor cells.
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PMID:[Immunohistochemical and ultrastructural studies of intracranial immature teratoma--a comparative observation on neuroblastoma, PNET and ependymal tumor, with a special reference to rosette structures]. 816 54

In a 15-year-old girl with right upper arm tumor diagnosed at first as neuroblastoma, the second histopathological examination revealed PNET. In spite of the advanced stage of disease (IIIrd or IVth) surgical excision was not performed but chemotherapy (8 courses of VADRAC and 8 courses of VP16 + CDDP) and radiotherapy (40 Gy) was administered. After 4 initial cycles all symptoms of the disease disappeared. The child remains in CCR for 60 months, including 40 months of therapy.
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PMID:[Malignant neuroectodermal tumor of the arm in a 15-year-old girl successfully treated with chemo- and radiotherapy]. 867 66

Fifteen children 4 years of age or under (8-46 months), weight 7.8 to 17 kg, underwent 44 peripheral blood stem cell (PBSC) collections. Diagnoses included PNET/medulloblastoma (five), neuroblastoma (five), and others (five). PBSCs were collected following G-CSF/GM-CSF or chemotherapy plus G-CSF/GM-CSF mobilization. All PBSC collections were well tolerated. The average yield per collection was 6.80 x 10(8) mononuclear cells/kg (1.1-30 x 10(8)/kg) or 57.60 x 10(6) CD34+/kg (1.37 to 480 x 10(6)/kg). Eight patients underwent stem cell transplantation following myeloablative chemotherapy. Six of the eight children who received PBSC following myeloablative therapy also received autologous bone marrow (0.7 to 3.6 x 10(8) MNC/kg). One heavily pretreated patient experienced delayed hematologic reconstitution, while the remaining seven patients had a median ANC recovery to > 0.5 x 10(3)/microliter by day +10 (9-11 days) and platelets > 50 x 10(3)/microliter by day +15 (12-17 days). Seven patients received PBSCs following repetitive submyeloablative chemotherapy (ICE: ifosfamide 1.8 g/m2/day, etoposide 100 mg/m2/day x 5, carboplatin 400 mg/m2/day x 2) or other similar combination chemotherapy. Median days to recover ANC > or = 1 x 10(3)/microliter and platelets > or = 100 x 10(3)/microliter in children receiving ICE + PBSCs were 10 and 14 days, respectively, compared with 16 and 22 days in children receiving ICE + G-CSF in historical controls. In conclusion, collection and use of PBSCs to support either myeloablative chemotherapy or multicycle submyeloablative chemotherapy is well tolerated and may enhance hematological recovery in young children and infants.
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PMID:Collection and use of peripheral blood stem cells in young children with refractory solid tumors. 902 45

The resting energy expenditure (REE) and the respiratory quotient (RQ) were measured longitudinally using indirect calorimetry to examine the effects of total parenteral nutrition (TPN) on energy metabolism in children undergoing autologous peripheral blood stem cell transplantation (PBSCT). There were six children (two males and four females) and the age ranged from five to 13 years (median, eight yrs). The diagnosis included acute lymphocytic leukemia (ALL; 4), neuroblastoma (NBL; 1) and primitive neuroectodermal tumor (PNET; 1). TPN was started after the patients were stabilized following PBSCT (group A; n = 3) or before the initiation of high-dose cytoreductive chemotherapy (HCC) (group B; n = 3). Duration of HCC before PBSCT was identical between the two groups (six to eight days). Average total calorie and protein intake during HCC was significantly higher for group B than for group A. The %REE, the percentage of REE to the predicted basal energy expenditure (BEE), in group A showed 133 +/- 19%, 129 +/- 14% and 146 +/- 11% during three periods of HCC (days -8 to -1 of PBSCT), bone marrow suppression (days 0 to 11 of PBSCT) and bone marrow recovery (days 12 to 22 of PBSCT), respectively. In contrast, those in group B were 10% to 20% lower than those in group A at all periods. Carbohydrate oxidation rates during HCC in group A were significantly lower than those in group B, and those were not different between both groups during post-PBSCT periods. Fat oxidation rates in both groups were similar at all stages of periods. In contrast, protein degradation rates in group A were significantly higher than those in group B at all stages of the period. From these results, we concluded that commencement of TPN administration prior to HCC in the patients undergoing PBSCT provides beneficial effects to maintain better energy metabolic and nutritional status.
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PMID:Total parenteral nutrition on energy metabolism in children undergoing autologous peripheral blood stem cell transplantation. 959 9

ILK (beta1-integrin-linked protein kinase) is a recently identified 59-kDa serine/threonine protein kinase that interacts with the cytoplasmic domain of the beta1-integrin containing four ankyrin-like repeats. We have developed a polyclonal antibody against ILK and explored the ILK immunoreactivity in normal human cells and tissues. ILK was mainly expressed in cardiac muscle and skeletal muscles. Surprisingly, ILK expression was observed in Ewing's sarcoma (ES; 100%), primitive neuroectodermal tumour (PNET; 100%), medulloblastoma (100%), and neuroblastoma (33.3%), whereas other small round cell sarcomas were not stained by the anti-ILK antibody. These results suggest that ILK could be a novel marker for tumours with primitive neural differentiation. Our findings support the notion that ES is a tumour that is closely related to PNET and that both originate from the neuroectoderm. ILK may be a sensitive and specific immunohistochemical marker and useful for the positive identification of ES and PNET in formalin-fixed, paraffin-embedded tissue sections.
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PMID:ILK (beta1-integrin-linked protein kinase): a novel immunohistochemical marker for Ewing's sarcoma and primitive neuroectodermal tumour. 973 88


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