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)

Various kinds of second malignant neoplasms after sucessful treatment for childhood acute leukemia have been reported. The authors describe an unusual case of an olfactory neuroblastoma in a patient previously treated for childhood acute leukemia including autologous bone marrow transplantation. The prophylactic cranial irradiation and the total body irradiation during autologous bone marrow transplantation may have induced the development of their patient's olfactory neuroblastoma. Although a second primary olfactory olfactory neuroblastoma is rare is rare, it should be added to the list of second malignant neoplasms in the sinonasal region.
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PMID:Olfactory neuroblastoma as a second malignant neoplasm in a patient previously treated for childhood acute leukemia. 1159 9

Eight children developed osteochondroma (OS) at a mean of 88 months after hematopoietic stem cell transplantation (HSCT). The mean age at HSCT was 56 months (12-84). This represents a cumulative incidence of 20% among patients less than 18 years of age transplanted from 1981 to 1997. These eight patients underwent allogeneic (n = 2) or autologous (n = 6) transplantation for either acute leukemia (n = 6) or neuroblastoma (n = 2) after a conditioning regimen including TBI (n = 7) or a combination of Bu and CY. OS was multiple in seven patients and solitary in one. Eight lesions were resected and all were benign. Four children received growth hormone before diagnosis of OS, but there was no clinical, radiological or histological difference between those who did not. Univariate analysis showed an increased rate associated only with autologous HSCT, with a 31.7% probability of a new OS at 12 years after HSCT. Osteochondroma should be added to the other adverse effects of HSCT in children.
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PMID:Osteochondroma after pediatric hematopoietic stem cell transplantation: report of eight cases. 1197 12

Aberrant promoter methylation of tumor suppressor genes has not been fully investigated in pediatric tumors. Therefore, we examined the methylation status of nine genes (p16(INK4A), MGMT, GSTP1, RASSF1A, APC, DAPK, RARbeta, CDH1 and CDH13) in 175 primary pediatric tumors and 23 tumor cell lines using methylation-specific PCR. We studied the major forms of pediatric tumors--Wilms' tumor, neuroblastoma, hepatoblastoma, medulloblastoma, rhabdomyosarcoma, osteosarcoma, Ewing's sarcoma, retinoblastoma and acute leukemia. The most frequently methylated gene in both primary tumors and cell lines was RASSF1A (40, 86%, respectively). However, the rates of RASSF1A methylation in individual tumor types varied from 0 to 88%. RASSF1A methylation was tumor specific and was absent in adjacent non-malignant tissues. Methylation of the other genes was relatively rare in tumors and non-malignant tissues (less than 5%). Neuroblastoma patients with methylation of RASSF1A were significantly older than patients without methylation (P=0.008). There was no relationship between methylation status and other clinico-pathologic parameters. We treated six cell lines lacking RASSF1A mRNA with 5-aza-2'deoxycytidine to examine the relationship between methylation and transcriptional silencing. In five of six cell lines, restoration of RASSF1A mRNA was confirmed by RT-PCR. Our findings indicate that aberrant promoter methylation of RASSF1A may contribute to the pathogenesis of many different forms of pediatric tumors.
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PMID:Aberrant promoter methylation and silencing of the RASSF1A gene in pediatric tumors and cell lines. 1208 24

A retrospective study of malignant diseases of childhood was carried out at B. S. Medical College, Bankura, W.B. to know the prevalence. It includes cases detected in this institution during 1990-1999 in the age group of 0-14 years. Cases were identified from previous records. Histopathology slides, stained by hematoxylin and eosin, were recovered and reviewed. Diagnosis were made by morphology. Hematological diagnosis were made by morphology and cyto-chemistry. In total 120 cases were detected. Acute leukemia (39.2%) was the commonest, followed by retinoblastoma (19.2%), lymphoma (10.8%), Wilm's tumor (10%) and rhabdomyosarcoma (9.1%). Brain tumors were not found due to non-availability of Neurosurgical Unit in this institution. Frequency of neuroblastoma was very low, only 2 out of a total of 120. In comparison to studies in Southern India and Western countries retinoblastoma and soft tissue sarcoma are more prevalent here, while there is a much lower prevalence of neuroblastoma.
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PMID:Childhood malignancies at BS Medical College: a ten year study. 1502 7

Calgary Laboratory Services (CLS) in Alberta, Canada, is the regional reference laboratory providing flow cytometry services for southern Alberta and southeastern British Columbia. As a busy reference flow laboratory we provide flow cytometry immunophenotyping for investigation and diagnosis of acute and chronic leukemias, lymphomas, immunodeficiencies, neuroblastoma, platelet disorders, and interstitial lung disease (ILD). Because of increasing workload and the continual effort to improve the service to our health care providers, CLS invested in the new Beckman Coulter Cytomics FC 500 5-color flow cytometer. In addition to time and labor savings due to reduced maintenance and operating system design, this new flow cytometer automates many of the previous manual steps involved in quality control and flow cytometric analysis. It also incorporates 2 lasers and is capable of measuring 5-color antibody combinations in a single tube, enabling us to reduce the number of tubes and overall costs, giving us better gating options for minimal residual disease analysis. We present the first published evaluation, an assessment of the overall productivity and cost impact of the new state-of-the-art Cytomics FC 500 flow cytometer. Implementation of the Cytomics FC 500 has resulted in a 20% reduction in reagent costs and shorter turnaround time for analysis and diagnosis. This instrument has allowed us to reduce our acute leukemia panel from 17 to 13 tubes, our lymphoma panel from 13 to 7 tubes, and our ILD panel from 4 to 2 tubes. The availability of 2 lasers provides more flexibility in choosing antibodies and conjugates to customize immunophenotyping panels. It also allows us to use the DRAQ5 dye and simultaneously analyze the immunophenotype and DNA content of cells with very little compensation. Many of the arduous, time-consuming flow operator tasks often associated with previous generation flow cytometry instruments, such as color compensation, list mode analysis, sample repeats, and interpretations, have been substantially reduced with the Cytomics FC 500 5-color flow cytometer. In conclusion the Cytomics FC 500 5-color flow cytometer is a major advance in flow cytometry instrumentation and has reduced our overall reagent costs by 20%, provided better information and speedier turnaround time to our health care professionals. It is an ideal flow cytometer for any busy clinical or research flow cytometry service.
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PMID:Impact of the new Beckman Coulter Cytomics FC 500 5-color flow cytometer on a regional flow cytometry clinical laboratory service. 1522 66

It has been suggested that breast milk may play a role in the prevention of certain childhood cancers. We undertook a systematic review of published studies investigating the association between breast-feeding and childhood cancers using Medline (1966 to June 2004), supplemented with auto alerts and manual searches. Analyses are based on odds ratios for specific cancers among those ever breast-fed compared with those never breast-fed, pooled using random-effects models. Forty-nine publications were potentially relevant; of these, 26 provided odds ratio estimates for at least one childhood cancer outcome and were included in metaanalyses. Overall, 92% of the studies were case-control studies, 85% relied on long-term recall of feeding history, only 8% examined breast-feeding exclusivity and control response rates were under 80% in over half. Metaanalyses suggested lower risks associated with having been breast-fed of 9% (95% CI = 2-16%) for acute lymphoblastic leukemia, 24% (3-40%) for Hodgkin's disease and 41% (22-56%) for neuroblastoma, with little between-study heterogeneity. The estimates for Hodgkin's disease and neuroblastoma, however, were driven by single studies. There was little evidence that breast-feeding was associated with acute nonlymphoblastic leukemia, non-Hodgkin's lymphoma, central nervous system cancers, malignant germ cell tumors, juvenile bone tumors, or other solid cancers. In conclusion, ever having been breast-fed is inversely associated with acute lymphoblastic leukemia, Hodgkin's disease and neuroblastoma in childhood, but noncausal explanations are possible. Even if causal, the public health importance of these associations may be small. Our estimates suggest that increasing breast-feeding from 50% to 100% would prevent at most 5% of cases of childhood acute leukemia or lymphoma. (c) 2005 Wiley-Liss, Inc.
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PMID:Breast-feeding and childhood cancer: A systematic review with metaanalysis. 1598 34

Cancer occurring in infants often has clinical and biological properties that are different from those of the same histologic type of cancer occurring in older children. The histologic distribution of cancers in infants and that in older children are also different. The aim of this study was to find these differences between infants and older children, and to compare the percent distribution of infant cancer subtypes with that reported by other countries. The authors collected infant cases diagnosed as having cancer from the database of the Cancer Registry in our Medical Center between 1995 and 2001. Subjects were selected subjects from inpatient logs, and their medical records were reviewed. Eighty-two infants (40 males and 42 females), including 12 neonates, were diagnosed with cancer over this 7-year period. Acute leukemia was diagnosed in 21 infants (25.6%; acute myeloid leukemia in 12, and acute lymphoblastic leukemia in 9), retinoblastoma in 14 (17.1%), neuroblastoma in 12 (14.6%), brain tumor in 9 (11.0%), germ cell tumor in 8 (9.8%), renal cancer in 8 (Wilms tumor 3, mesoblastic nephroma 1, renal sarcoma 1, rhabdoid tumor 3), hepatoblastoma in 5 (6.1%), and soft tissue sarcoma in 5 (rhabdomyosarcoma 1, fibrosarcoma 3, other sarcoma 1). The overall disease-free survival rate was 61.0% (50/82) with a median follow-up duration of 6.8 years for the survivors. The 4 most common types of cancer occurring in infants are the same in the present series and in most larger childhood cancer series reported by other countries; but rank differently. In this study there were more infants with acute leukemia and retinoblastoma, and less with neuroblastoma. The prognosis is poor for infant leukemia and rhabdoid tumor, while it is good for embryonal tumors and germ cell tumors occurring in infancy.
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PMID:Cancer in infants: a review of 82 cases. 1616 13

Cancer vaccines are examples of active immunotherapy. In pediatric malignancy such active strategies may be particularly problematic because of immune suppression produced by the tumor or its intensive treatment with combined chemotherapy. Nonetheless, the expression of tumor-specific and tumor-associated antigens on a range of pediatric tumors has encouraged investigation of the approach in patients with either bulky or minimal residual disease. Here we describe promising results in neuroblastoma and acute leukemia, suing genetically modified whole cell vaccines, peptides, and dendritic cells. The difficulties of conducting and evaluating such studies in a pediatric population are also described, and a strategy for cancer vaccine development is outlined.
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PMID:Vaccine therapies for pediatric malignancies. 1619 23

Parents asked to consent to a child's randomization in a pediatric cancer clinical trial are often unprepared to grasp the implications of this scientifically crucial but seemingly unfair process. Physicians must adopt specific communication skills to engage families in open dialogue from the outset in order to elicit truly shared informed consent. Starting from the case of a family with an only child affected by disseminated neuroblastoma, we wish to comment on the problems surfacing in the informed consent process for treatment and research in pediatric oncology that implicate an understanding of bioethical issues and psychological principles. Although the outcome of childhood cancer has improved dramatically over the last 30 years, with overall survival rates now exceeding 70%, there are regretfully still types and stages of cancer carrying a very high risk of death that urgently require new clinical strategies. The response to this need has been the design of experimental protocols that often entail randomized controlled trials (RCT). A large number of these trials concern stage IV neuroblastoma, acute leukemia, rhabdomyosarcoma, and other types of childhood cancers presenting great heterogeneity both in terms of localization and responsiveness to therapy. Most trials for disease relapses also include one or more randomizations. The scientific motivation justifying an RCT is the need to compare and evaluate an innovative protocol (or part thereof) with reference treatment modalities. Nevertheless, the process brings to bear the ethical dilemma of having to weigh the needs of the single afflicted child against the benefit which may ensue for a much larger patient community.
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PMID:Randomization, informed consent and physicians' communication skills in pediatric oncology: a delicate balance. 1639 46

Noonan syndrome (NS) is an autosomal dominant disorder characterized by short stature, typical craniofacial dysmorphism, skeletal anomalies, congenital heart defects, and predisposition to malignant tumors. In approximately 50% of cases, the disease is caused by missense mutations in the PTPN11 gene. To date, solid tumors, and particularly brain tumors and rhabdomyosarcomas, have been documented in patients with NS; however, few cases of neuroblastoma associated with NS have been reported. Here we report an unusual case of neuroblastoma with mediastinal, retroperitoneal, and medullar locations associated in a NS patient carrying a PTPN11 germline missense mutation (p.G60A). This missense mutation occurs within the N-SH2 domain of the PTPN11 gene and has been reported to be associated with acute leukemia in NS patients. The association of this p.G60A PTPN11 mutation with neuroblastoma provides new evidence that gain of function PTPN11 mutations may play an important role in the pathogenesis of solid tumors associated with Noonan syndrome.
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PMID:Germline PTPN11 missense mutation in a case of Noonan syndrome associated with mediastinal and retroperitoneal neuroblastic tumors. 1832 49


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