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)

Positron emission tomography/computed tomography (PET/CT) has become an important imaging modality in the non-invasive evaluation and monitoring of children with known or suspected malignant diseases. In sarcoma patients, [18F]FDG (FDG) PET and FDG PET/CT is useful in staging, therapy monitoring, and detection of relapse. However, FDG PET has been proven to be less sensitive than chest CT in the detection of pulmonary metastases derived from sarcoma. This disadvantage has been overcome using a PET/CT scanner. In neuroblastoma patients, PET using FDG is indicated in MIBG-negative cases. Furthermore, there are specific PET tracers for tumors of the sympathetic nervous system, such as [11C]Hydroxyephedrine (HED) and [18F]-labeled dihydrophenylalanine (F-DOPA), which can be used for PET/CT imaging for detection of disease, staging and monitoring therapy. However, there are only few studies using specific PET tracers in neuroblastoma patients. In other pediatric malignancies including germ cell tumors and hepatoblastoma PET and PET/CT may be helpful in individual cases, but the literature in these entities is limited so far. Although publications on the additional value of the combined PET/CT compared to both stand-alone modalities are still limited in pediatrics, it can already be anticipated that the combination of morphological and functional information obtained by integrated PET/CT will improve the accuracy of staging and will change patient management in a significant number of pediatric patients.
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PMID:FDG-PET/CT in pediatric solid tumors. 2082 8

The Hedgehog (Hh) signaling pathway regulates the development of many organs in mammals. Recent studies have indicated that the activation of the Hh signaling pathway contributes to the growth of various adult cancers. However, little is known about its role in the development of pediatric malignancies. The present study was undertaken to examine the expression and functional involvement of Hh signal transcription factors in pediatric tumor cells in order to determine their potential as therapeutic targets. We utilized real-time RT-PCR to investigate the expression of Glioma-associated oncogene homolog 1 (Gli1) in various pediatric tumor cell lines, including rhabdomyosarcoma, neuroblastoma and hepatoblastoma. The mRNA expression of Gli1 was markedly increased in rhabdomyosarcoma (RMS-YM, RD, RH30) cell lines, and moderately increased in neuroblastoma (NB19) and hepatoblastoma (Huh6) cell lines. The proliferation of these cell lines was dose dependently inhibited by Forskolin, a specific Hh signal inhibitor. In addition, Forskolin-induced growth suppression was associated with the down-regulation of C-Myc. Moreover, the blockade of Hh signaling with Forskolin enhanced cell apoptosis in a dose-dependent manner. These results demonstrated that Hh signal activation frequently occurs in neuroblastoma, hepatoblastoma and rhabdomyosarcoma cell lines. The inhibition of Hh signaling suppressed proliferation and increased apoptosis in these tumor cells. These findings suggest that the Hh signaling pathway plays an important role in tumorigenesis and is a potential molecular target of new treatment strategies for these pediatric malignant tumors.
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PMID:Forskolin, a Hedgehog signal inhibitor, inhibits cell proliferation and induces apoptosis in pediatric tumor cell lines. 2147 12

Fucoidan, the general term for sulfated polysaccharides, is reported to engage in various biological activities having anti-tumor, anti-coagulation and anti-viral effects. Though it has been investigated, the mechanism of its anti-tumor effects remains elusive. The current study examined the anti-tumor effects of fucoidan extracted from Okinawa mozuku on 15 human cancer cell lines (6 hepatocellular carcinomas, 1 cholangiocarcinoma, 1 gallbladder cancer, 2 ovarian cancers, 1 hepatoblastoma, 1 neuroblastoma and 3 renal cancers) using an MTT assay. Changes in apoptosis and the cell cycle were analyzed by flow cytometry. The results revealed that cell proliferation was suppressed in 13 cell lines in a time- and/or dose-dependent manner; this suppression was marked in the hepatocellular carcinoma, cholangiocarcinoma and gallbladder carcinoma cell lines. In contrast, proliferation of the neuroblastoma and 1 of the 2 ovarian carcinoma cell lines was not affected. The ratio of apoptotic cells significantly increased in 5 of the 6 hepatocellular carcinoma cell lines, and the ratio of G2/M cells increased in the 3 hepatocellular cell lines examined. These observations indicate that fucoidan is a potential anti-tumor agent for the treatment of bile duct cancers, such as hepatocellular carcinoma, cholangiocarcinoma and gall-bladder carcinoma.
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PMID:Fucoidan, a major component of brown seaweed, prohibits the growth of human cancer cell lines in vitro. 2147 46

Malignant small round cell tumors are characterised by small, round, relatively undifferentiated cells. They generally include Ewing's sarcoma, peripheral neuroectodermal tumor, rhabdomyosarcoma, synovial sarcoma, non-Hodgkin's lymphoma, retinoblastoma, neuroblastoma, hepatoblastoma, and nephroblastoma or Wilms' tumor. Other differential diagnoses of small round cell tumors include small cell osteogenic sarcoma, undifferentiated hepatoblastoma, granulocytic sarcoma, and intraabdominal desmoplastic small round cell tumor. Differential diagnosis of small round cell tumors is particularly difficult due to their undifferentiated or primitive character. Tumors that show good differentiation are generally easy to diagnose, but when a tumor is poorly differentiated, identification of the diagnostic, morphological features is difficult and therefore, no definitive diagnosis may be possible. As seen in several study reports, fine needle aspiration cytology (FNAC) has become an important modality of diagnosis for these tumors. The technique yields adequate numbers of dissociated, viable cells, making it ideally suitable for ancillary techniques. Typically, a multimodal approach is employed and the principal ancillary techniques that have been found to be useful in classification are immunohistochemistry and immunophenotyping by flow cytometry, reverse transcriptase polymerase chain reaction (RT-PCR), fluorescence in situ hybridization (FISH), and electron microscopy. However, the recent characterization of chromosomal breakpoints and the corresponding genes involved in malignant small round cell tumors means that it is possible to use molecular genetic approaches for detection.
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PMID:Malignant small round cell tumors. 2193 41

Embryonal cancers are a heterogeneous group of rare cancers which mainly occur in children and adolescents. The aim of the present study was to estimate the burden (incidence, prevalence, survival and proportion of cured) for the principal embryonal cancers in Europe (EU27), using population-based data from cancer registries (CRs) participating in RARECARE. We identified 3322 cases diagnosed from 1995 to 2002 (latest period for which data are available): 44% neuroblastoma, 35% nephroblastoma, 13% retinoblastoma and 6% hepatoblastoma. Very few cases of pulmonary blastoma (43 cases) and pancreatoblastoma (seven cases) were diagnosed. About 2000 new embryonal cancers were estimated every year in EU27, for an annual incidence rate of 4 per million (1.8 neuroblastoma, 1.4 nephroblastoma, and 0.5 retinoblastoma); 91% of cases occurred in patients under 15 years. Five-year relative survival for all embryonal cancers was 80% (99% retinoblastoma, 90% nephroblastoma, 71% hepatoblastoma and 68% neuroblastoma). Overall survival was lower in adolescents and adults than in those under 15 years. The cure rate was estimated at 80%. Slightly less than 40,000 persons were estimated alive in EU27 with a diagnosis of embryonal cancer in 2008. Nephroblastoma was the most prevalent (18,150 cases in EU27), followed by neuroblastoma (12,100), retinoblastoma (5200), hepatoblastoma (2700) and pulmonary blastoma (614). This is the first study to delineate the embryonal cancer burden in Europe by age, sex and European region. Survival/cure rate is generally high, but there are considerable gaps in our understanding of the natural histories of these rare diseases particularly in adults.
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PMID:Embryonal cancers in Europe. 2235 15

This study was aimed to investigate the expression of ICAM-1 (CD54) in pediatric tumor and acute leukemia (AL), so as to understand the distribution of ICAM-1 and its clinical significance. The expression of ICAM-1 in tissues of 46 pediatric tumor patients were detected by immunohistochemistry, and in bone marrow cells of 60 pediatric acute leukemia (AL) patients were detected by flow cytometry. 46 pediatric tumor patients included 10 lymphoma, 3 hepatoblastoma, 6 neuroblastoma, 2 rhabdomyosarcoma, 6 Ewing's bone sarcoma, 2 fibrosarcoma, 5 primitive neuroectodermal tumor, 11 nephroblastoma and 1 osteosarcoma. 60 AL pediatric patients included 20 acute lymphocytic leukemia (ALL) patients and 40 acute nonlymphocytic leukemia (ANLL) patients containing 20 M1, M2, M3 patients and 20 M4, M5. The results indicated that expression of ICAM-1 was more positive in all 3 hepatoblastoma cases, which represent a higher positive rate than that in lymphoma, neuroblastoma, rhabdomyosarcoma, Ewing's sarcoma of bone and osteosarcoma. However, no expression of ICAM-1 was observed in fibrosarcoma, nephroblastoma and primitive neuroectodermal tumor patients. On the other hand, the expression rate of ICAM-1 was 55 in ALL, 65 in ANLL M1, M2, M3, and 50 in ANLL M4, M5. It is concluded that the expression of ICAM-1 in pediatric tumor and AL has variability. The ICAM-1 positive expression is observed in hepatoblastoma and ANLL M1, M2, M3 patients, whereas it is undetectable in fibrosarcoma, nephroblastoma and primitive neuroectodermal tumor patients.
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PMID:[Expression of ICAM-1 (CD54) in pediatric tumor and acute leukemia and its clinic significance in immunotherapy with CIK cell]. 2254 Oct 82

Common pediatric malignancies are reviewed: neuroblastoma, Wilms tumor, hepatoblastoma, rhabdomyosarcoma, and sacrococcygeal teratoma. Elements of presentation, diagnosis, staging, treatment, and longterm prognosis are discussed, with particular attention to surgical management.
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PMID:Pediatric malignancies: neuroblastoma, Wilm's tumor, hepatoblastoma, rhabdomyosarcoma, and sacroccygeal teratoma. 2259 19

Tumor predisposition in children is rare, accounting for approximately 10% of all cancers in childhood. Tumor predisposition involves very rare tumors such as pleuropulmonary blastoma, adrenocortical carcinoma, hepatoblastoma, rhabdoid tumors, optic pathway glioma, as well as rare tumors such as retinoblastoma, medulloblastoma, nephroblastoma, or more frequent tumors such as sarcomas, neuroblastoma, and leukemias. The identification of these predispositions is important for improved management for both the child and relatives. Prenatal and preimplantation genetic diagnosis are options that could be considered for young parents in a perspective of future pregnancies. This manuscript describes the main tumor predispositions in childhood. From each histological subtype, the different diagnosis directions are discussed in view of these main tumor predispositions.
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PMID:[Genetic predisposition to childhood cancer]. 2279 86

Glypican-3 (GPC3) is a proteoglycan thought to play an important role during development. Germline GPC3 mutations are seen in the rare Simpson-Golabi-Behmel syndrome (SGBS), which predisposes patients to Wilms tumor, hepatoblastoma, and neuroblastoma. While numerous adult tumors have been evaluated by immunohistochemistry for GPC3, no comprehensive assessment has been done in pediatric tumors. We therefore investigated GPC3 expression in 143 pediatric central nervous system (CNS) tumors and 271 non-CNS tumors. Among non-CNS tumors, GPC3 expression was seen in 9/9 (100%) hepatoblastomas, 4/6 (67%) malignant rhabdoid tumors, 5/13 (38%) Wilms tumors, 11/37 (30%) alveolar rhabdomyosarcomas, and 8/45 (18%) embryonal rhabdomyosarcomas. All 136 neuroblastomas, 14 Ewing sarcoma/primitive neuroectodermal tumors, and 11 synovial sarcomas were immunonegative for GPC3. Among CNS tumors, GPC3 had restricted expression, with positivity in 6/6 (100%) atypical teratoid rhabdoid tumors and 1/4 (25%) craniopharyngiomas. The remaining 136 CNS tumors-23 medulloblastomas, 21 pilocytic astrocytomas, 13 gangliogliomas, 12 ependymomas, 12 glioblastomas, 11 choroid plexus neoplasms, 10 diffuse astrocytomas (grade II/III), 10 meningiomas, 8 dysembryoplastic neuroepithelial tumors, 8 oligodendrogliomas, 3 craniopharyngiomas, 3 germinomas, and 2 neurocytomas-were entirely negative for GPC3. These results showed GPC3 positivity in a number of non-CNS tumors, with no consistent discrimination between tumors that were or were not associated with SGBS. Within the CNS, GPC3 positivity was limited to a small subset of CNS neoplasms and may thus serve as a useful positive diagnostic biomarker (P < 0.0001) in addition to negative INI1/BAF47/SMARCB1 staining to differentiate atypical teratoid rhabdoid tumors from other high-grade pediatric brain tumors.
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PMID:Immunohistochemical expression of glypican-3 in pediatric tumors: an analysis of 414 cases. 2353 Sep 9

There is little data on the amount of time patients and families typically wait for pathology results when pediatric malignancy is suspected. The purpose of this study was to determine the average waiting period after diagnostic intervention for pediatric cancer. Pathology reports were reviewed for pediatric patients who had their initial diagnosis and were followed in the Division of Hematology/Oncology from 2007 through 2010. The average turnaround time (TAT) for all pathology (n=266) was 6.9 days. The TAT for pathology results according to diagnosis was 10.1 days for CNS tumors (n=59), 9.7 days for sarcomas (n=40), 5.4 days for lymphomas (n=31), 5.4 days for neuroblastoma (n=13), 7.3 days for kidney tumors (n=11), 7.2 days for thyroid tumors (n=7), 9.4 days for ovarian tumors (n=7), 7.0 days for schwannomas/neurofibromas (n=5), 5.7 days for testicular tumors (n=3), 5.0 days for hepatoblastoma (n=3), and 7.0 days for nasopharyngeal carcinomas (n=2). Overall the TAT for leukemia was 3.1 days (n=76), with diagnosis by flow cytometry taking 1.2 days and results by bone marrow biopsy taking 4.0 days. The TAT for pediatric oncology pathology after diagnostic intervention varies according to diagnosis. The hope is that this information will better prepare patients and families for the agonizing waiting period associated with diagnosis.
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PMID:Pathology turnaround time in pediatric oncology: a tool to prepare patients and families for the diagnostic waiting period. 2382 14


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