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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of abdominal Burkitt's lymphoma diagnosed through aspiration cytology is described. This 9-year-old boy presented with abdominal pain and distention for three months accompanied by fever and night sweat during the last month. An abdominal sonography and CT scan showed hepatosplenomegaly and an intrahepatic mass with celiac lymph node enlargement, ascites, and pleural fluid. A peripheral blood smear showed a few blast cells. Aspiration of the abdominal mass revealed very cellular aspirates consisting of diffusely scattered small monotonous round cells. The cells had little cytoplasm, along with round nuclei that showed clear-cut nuclear membrane, coarse chromatin pattern, and multiple small prominent nucleoli. Differential diagnoses considered were small round cell sarcomas such as malignant lymphoma, neuroblastoma, Ewing's sarcoma, and rhabdomyosarcoma. Of these, malignant lymphoma of the small noncleaved cell type was most consistent with the results of several studies including immunohistochemical staining, peripheral blood smear, and bone marrow biopsy. The cells were positive for leukocyte common antigen (LCA) and showed finely vacuolated basophilic cytoplasm in both the peripheral blood smear and bone marrow biopsy, characteristic of Burkitt's lymphoma cells.
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PMID:Abdominal Burkitt's lymphoma diagnosed by fine needle aspiration cytology--a case report. 227 68

The features of neuroblastoma and outcome of its management in 15 Nigerian children seen in the Ahmadu Bello University Teaching Hospital, Zaria over a 7-year period are described. The majority were between the ages of 3 and 5 years and the male:female ratio was 2.75:1. Over half of the patients presented with abdominal masses, about half with proptosis and a third with skull nodules. The diagnosis of neuroblastoma was correctly made on admission in only five instances. The mainstay of treatment was chemotherapy and the outcome was uniformly poor. Only one patient attended follow-up for as long as 1 year. Based on the present study, the frequency of neuroblastoma ranks third, after Burkitt's lymphoma and Wilms' tumour, amongst malignant childhood solid tumours encountered in the paediatric unit of Ahmadu Bello University Teaching Hospital. The low index of suspicion and poor diagnostic facilities might, however, have contributed to the low reported incidence, late diagnosis and poor survival rates of children with this tumour.
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PMID:Neuroblastoma, an under-diagnosed tumour: a 7-year experience in Zaria. 246 Nov 49

Data from the Papua New Guinea Tumour Registry and the Central Pathology Department were reviewed in order to document the incidence and pattern of malignancies in children in Papua New Guinea. Altogether, 680 cases of histologically defined childhood malignancies were recorded during the 14.5 years from 1971 to 1985. The frequencies of the various tumours were compared with past data and with published data from other countries. The incidence of malignancies in Papua New Guinean children appeared to be low, 36.5/1,000,000/year, with a male:female ratio of 1.6:1. Lymphoma was the most commonly occurring tumour and Burkitt's tumour accounted for 53% in this group. The relative frequency of leukaemia compared with lymphoma appeared to have increased since a previous report. A relatively high incidence of retinoblastoma (6.9%) and of other embryonal tumours (4.8%) was recorded, whilst the recorded incidences of tumours of the central nervous system (3.8%) and neuroblastoma (3.7%) were low. Ewing's sarcoma accounted for almost half of the bone tumours, whilst Kaposi's sarcoma was a relatively frequent soft tissue tumour. Differences and similarities between the Papua New Guinea data and those from other countries are discussed.
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PMID:Childhood malignant tumours in Papua New Guinea. 246 3

In the period 1985-88, 171 fine needle aspirates from paediatric patients with malignant and non-neoplastic masses were processed and evaluated in the Department of Haematology, Kenyatta National Hospital, Nairobi. Sixty-five needle aspirates had the diagnosis corroborated by histological reports. The rest had relevant clinical and laboratory information to support the cytological diagnosis. The histological diagnosis confirmed cytological diagnosis in 100% for neuroblastoma, 96% for Burkitt's lymphoma, 75% for carcinoma, 68% for sarcoma cases, 53% non-Hodgkin's lymphoma and 50% for Hodgkin's lymphoma. There were no false positives. It is therefore concluded that fine needle aspiration is a useful tool. It may obviate diagnostic surgery, help in planning the course of management of patients and it is diagnostic in Burkitt's lymphoma and neuroblastoma. Fine needle aspiration cytology is an easy, cheap and quick investigation compared to surgical biopsy.
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PMID:The role of needle aspiration cytology in the differential diagnosis of accessible paediatric tumours. 259 25

In view of the personal observation that malignant peripheral neuroectodermal tumours (MPNT) can present different histological growth patterns, 41 cases of MPNT were histologically and immunohistochemically studied. The median age of the 41 patients was 15 years (range: 9 months - 23 years). There were 27 males and 14 females. Most tumours (23/41) were located in the thoracopulmonary region. In 31/41 cases there was bone as well as soft tissue involvement. The following histopathological patterns were found: Ewing's sarcoma-like (n = 7), atypical Ewing's sarcoma-like (n = 4), neuroblastoma-like (n = 8), rhabdomyosarcoma-like (n = 8), and hemangiopericytoma-like (n = 1). In 2 cases combined patterns were noted, one tumour being characterized by neuroblastoma-like and Burkitt's lymphoma-like features. Most cases of MPNT differed from the cytological features of typical Ewing's sarcoma in that they contained hyperchromatic nuclei with distinct nucleoli. Some reticulin fibrils were found in between the cells of some cases. Immunohistochemically, 19/23 cases reacted positively to vimentin, 29/32 to neuron specific enolase (NSE), 16/28 to protein S-100, and 1/9 to glial fibrillary acidic protein. 12/24 cases reacted positively to NSE and protein S-100. Neurofilaments and desmin were not found in the formalin fixed material of the present study. The results show that most cases of MPNT can be distinguished from typical Ewing's sarcoma by cytological and histological findings. Differential diagnosis from atypical Ewing's sarcoma, neuroblastoma, and rhabdomyosarcoma is possible by immunohistochemistry.
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PMID:[Malignant peripheral neuroectodermal tumors. Histological and immunohistological conditions in 41 cases]. 267 76

Using experimental modes with normal allogeneic bone marrow (BM) contaminated with Burkitt or neuroblastoma cell lines, and a liquid culture assay, we demonstrated that, when used in optimal conditions, the immunomagnetic depletion technique permitted a reproducible elimination of 3-4 log malignant cells. Results were very similar to those obtained with the complement lysis procedure in Burkitt lymphoma. This immunomagnetic procedure was used in 123 cases of autologous bone marrow transplantation (ABMT) in children with neuroblastoma. The analysis of the cases demonstrated, first, that the procedure induced a significant loss of mononuclear cells but was not toxic for BM precursors. Delays to engraftment observed in a few patients were probably due to the combination of pejorative factors, especially the damage caused to the micro-environment by previous heavy and prolonged chemotherapy or the double ABMT programme. Second, patients presented with profound T-cell defect with undetectable IL2 secretion up to 1 year post-graft but they all had normal NK functions from the first month post-graft, these functions exceeding normal values on the second and third months post-graft. Finally, in 20 cases, dual-immunofluorescence staining permitted the demonstration that the autograft contained malignant cells before purging that were eliminated by the immunomagnetic depletion.
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PMID:Immunomagnetic depletion of malignant cells from autologous bone marrow graft: from experimental models to clinical trials. 268 82

This report is a retrospective review of early studies (1965-70) on the cytology and short-term tissue culture of fresh specimens from 1643 patients under investigation for cancer at the University College Hospital, Ibadan. A total of 580 specimens were positive for malignant cells. The technical procedures are described in some detail and were found to be particularly useful as aid to laboratory diagnosis of 310 childhood tumours. The relative frequency of Burkitt's lymphoma, retinoblastoma, neuroblastoma and Wilm's tumour in the series was 20:1:1:0.5 respectively. Adoption of the technique as part of routine diagnostic service of teaching hospitals of developing countries is recommended.
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PMID:Cytopathology and short-term culture of malignant tumours in Ibadan. 299 44

Altered structure and regulation of the c-myc proto-oncogene have been associated with a variety of human tumours and derivative cell lines, including Burkitt's lymphoma, promyelocytic leukaemia and small cell lung cancer (SCLC). The N-myc gene, first detected by its homology to the second exon of the c-myc gene, is amplified and/or expressed in tumours or cell lines derived from neuroblastoma, retinoblastoma and SCLC. Here we describe a third myc-related gene (L-myc) cloned from SCLC DNA with homology to a small region of both the c-myc and N-myc genes. Human genomic DNA shows an EcoRI restriction fragment length polymorphism (RFLP) of L-myc defined by two alleles (10.0- and 6.6-kilobase (kb) EcoRI fragments), neither associated disproportionately with SCLC. Mouse and hamster DNAs exhibit a 12-kb EcoRI L-myc homologue, which indicates conservation of the gene in mammals. Gene mapping studies assign L-myc to human chromosome region 1p32, a location distinct from that of either c-myc or N-myc but associated with cytogenetic abnormalities in certain human tumours. This L-myc sequence is amplified 10-20-fold in four SCLC cell line DNAs and in one SCLC tumour specimen taken directly from a patient. Either the 10.0- or 6.6-kb allele can be amplified and in heterozygotes only one of the two alleles was amplified in any SCLC genome. SCLC cell lines with amplified L-myc sequences express L-myc-derived transcripts not seen in SCLC with amplified c-myc or N-myc genes. In addition, some SCLCs without amplification also express L-myc-related transcripts. Together, these findings suggest an enlarging role for myc-related genes in human lung cancer and provide evidence for the concept of a myc family of proto-oncogenes.
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PMID:L-myc, a new myc-related gene amplified and expressed in human small cell lung cancer. 299 22

The human B-cell line RJ2.2.5, derived by mutagenesis from a Burkitt lymphoma cell line and selected for loss of HLA class II antigen expression, was infected with recombinant retroviruses containing either the Harvey murine sarcoma virus oncogene v-Ha-ras or the human neuroblastoma homolog NRAS. Both activated ras genes partially complemented the regulatory defect in RJ2.2.5 and specifically increased the expression of the DR and DQ subsets of HLA class II genes. Blot-hybridization analysis and RNase mapping indicated that HLA-DQ alpha-chain mRNA in the infected cell lines was increased to a level at least 50% that of the parent B-cell line, Raji. The levels of HLA-DR and -DQ beta-chain RNA also were increased but to a lesser extent. In contrast, we detected no effect of ras on the quantities of other class II, class I, or invariant-chain mRNAs. Fluorescence-activated cell sorter analysis with antibodies recognizing HLA-DR, -DQ, and class I antigens supported these observations. Enhancement of HLA class II gene expression by ras genes may have important implications for regulation of the immune system in response to transformation.
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PMID:Defective HLA class II expression in a regulatory mutant is partially complemented by activated ras oncogenes. 331 16

It is clear that there are at least two classes of cancer-related genes. The more characterized of these are the oncogenes, whose activation appears to play a major role in human neoplasia. There are now two families of oncogenes, the myc and ras families, whose cooperation seems capable of transforming normal cells in culture to tumorigenic cells. As such, they appear to form complementation groups with immortalizing and transforming properties, respectively. Moreover, the oncogenes can be subclassified as tyrosine kinases or kinase related, GTP binding proteins, growth factors or growth factor receptors or nuclear proteins. More than 20 viral oncogenes have been identified, for which more than 30 proto-oncogenes or pseudogenes exist in the human genome. Many of these have been cloned, characterized to some extent, and mapped to particular chromosomes or regions of chromosomes. Further, more than 20 additional putative oncogenes or transforming genes have been identified by tumor DNA transfection studies or at sites of integration or translocation for which no viral transforming gene cognates exist. Oncogenes can be activated by increased or unregulated expression, increased copy number (duplication, amplification), or somatic mutation resulting in a protein with increased oncogenic potential. Examples of all of these mechanisms can be found in several specific human cancers or leukemias. The cytogenetic correlate of enhanced expression is a translocation between two chromosomes at specific breakpoints with no net loss of genetic material (e.g., increased c-myc expression resulting from the 8;14 translocation in Burkitt's lymphoma). The phenomenon of increased gene copy number can sometimes be visualized as trisomy or tetrasomy for a particular chromosome but more dramatically as the development of extrachromosomal DMs or as chromosomally integrated HSRs (e.g., the N-myc gene amplification seen in neuroblastoma). Finally, certain somatic mutations can be associated with translocations (e.g., the bcr/abl fusion product created as a result of the 9;22 translocation in chronic myelogenous leukemia), but they are more commonly submicroscopic (as characterized by point mutations in the ras gene family). Evidence is accumulating for a second class of cancer-related genes whose absence or inactivation is associated with tumorigenesis. These genes are associated at the cytogenetic level with chromosomal deletions, in which the breakpoints may be variable, but specific, common regions are consistently deleted.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:The involvement of oncogenes and suppressor genes in human neoplasia. 331 93


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