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

Seventeen patients with olfactory neuroblastoma seen at the Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary from 1941 to 1971 are presented. Data on age, sex, symptoms, physical, and roentgenographic findings, and therapeutic results are evaluated. A system of pretherapy staging is proposed in which for Group A, the tumor is limited to the nasal cavity; in group B, the tumor is localized to the nasal acvity and paranasal sinuses; and in group C, the tumor extends beyond the nasal cavity and paranasal sinuses. Olfactory neuroblastoma is a radioresponsive, and, to a limited extent, radiocurable tumor and varies in aggressiveness. Of 17 patients in this series, 13 or 76% were alive without disease following treatment by surgery, irradiation, and combination of these two methods. Uncontrolled primary lesions with or without metastases accounted for all therapeutic failures. A treatment policy for this disease is presented.
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PMID:Olfactory neuroblastoma. A clinical analysis of 17 cases. 126 Jun 76

A peculiar human cell line (GI-ME-N) derived from the metastatic bone marrow of a 2-yr-old patient with stage IV neuroblastoma (NB) was extensively characterized. Cell-type-specific markers, tumorigenicity in nude mice, morphology, cytogenetics, and amplification/expression of the N-myc gene were evaluated. All metaphases presented the typical 1p deletion. Surface markers specific for NB cells, vimentin, and neurofilament proteins were all clearly detectable with immunofluorescence and/or western blot procedures. Moreover, it was found that GI-ME-N cells did not express N-myc oncogene or HLA class 1 antigens, and were not classified as peripheral neuroectodermal tumor cells. However, extremely short latency and survival times, comparable to peripheral neuroectodermal tumor cells, were observed in nude mice grafted with GI-ME-N. In addition, no correlations were observed in tumorigenicity of N-myc amplified (IMR32) versus unamplified (SK-N-SH GI-ME-N) human NB cell lines in nude mice. We conclude that N-myc amplification/expression do not correlate with the aggressiveness of human NB in athymic animals, which is not always explained by the peripheral neuroectodermal tumor cell nature of the malignant cells, either.
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PMID:A new human highly tumorigenic neuroblastoma cell line with undetectable expression of N-myc. 229 63

Diploid DNA content, advanced stage, unfavorable histology, and N-myc amplification are all associated with aggressive disease and poor prognosis in childhood neuroblastoma. DNA diploidy is associated with advanced stage and unfavorable histology, but the relationships among ploidy, N-myc amplification, and proliferative activity are not known. To determine if DNA diploidy is associated with N-myc amplification, we studied 29 neuroblastomas with flow cytometric analysis and Southern blot analysis. Clinical and histologic features were also evaluated. Sixty percent of the N-myc-amplified tumors were diploid, compared to 26% of the neuroblastomas, which lacked N-myc amplification (P = 0.11). In our analysis of proliferative activity and N-myc amplification, a higher mean percentage of cells in S phase was seen in the N-myc-amplified tumors (13.4%) than in the unamplified tumors (10%), but again the result was not statistically significant (P = 0.14). Significant associations were seen between unfavorable histology and DNA diploidy (P = 0.05), and between unfavorable histology and high proliferative activity (P = 0.007). Our data suggest that biologic factors other than N-myc amplification play a role in determining the aggressiveness of at least some diploid neuroblastomas.
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PMID:Analysis of DNA ploidy and proliferative activity in relation to histology and N-myc amplification in neuroblastoma. 234 63

The rapid progress in molecular biology has allowed investigators to define some of the basic mechanisms of carcinogenesis. At the molecular level, cellular transformation results from the occurrence of two or three distinct genetic events that uncouple the cell from its normal regulatory mechanisms. One family of genes, the oncogenes, may be particularly important in the process. The aberrant expression of oncogenes, either by mutation or simply by increased transcription, may result in cellular transformation. The genes usually code for growth factors, growth factor receptors or for proteins involved in the transduction of growth signals into the nucleus. Genetic activation causes the cell to continuously sense a message to undergo mitosis, and the cell no longer responds to its normal regulatory signals. The concepts are rapidly moving into the clinical realm as the genetic mechanisms of particular neoplasms have been investigated. The neuroblastoma is the first tumor system in which the biologic characteristics of the tumor were found to be related to a known oncogene; the amplification of the myc gene is an independent marker of the aggressiveness of the tumor. In addition, much progress has been made in defining the specific genetic lesions responsible for retinoblastomas, Wilms' tumors and carcinomas of the colon that arise in patients with familial polyposis coli. As more knowledge accumulates regarding the exact mechanisms through which the proteins encoded by oncogenes affect these carcinomas and others, it may become possible to design pharmacologic agents rationally to hinder their growth selectively.
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PMID:The emerging genetics of cancer. 264 25

A large number of studies have investigated the relationship between the long-term survival and the percentage of tumor cells in S phase assessed by autoradiography after tritiated thymidine labelling, image cytometry, flow cytometry or labelling with an halogenated analog of thymidine, in various types of human solid tumors. The survey of the results clearly shows that the S-phase fraction (SPF) is of high prognostic significance in several types of cancers, in particular in breast cancers, non-Hodgkin lymphomas, ovarian cancers, neuroblastoma, bladder cancers and lung cancers. SPF was found of high independent significance in 10 of the 11 studies in which multivariate analyses of prognostic factors had been carried out. Proliferation appears generally to be of higher prognostic significance than ploidy. In view of the wide differences in the biological characteristics of the tumors studied, it is likely that the association between a high proliferation rate and the degree of tumor aggressiveness is a general feature of human solid tumors. However, high proliferative rate of tumor cells is probably not the cause of tumor biological aggressiveness but a variable associated with it. The extent to which cells escape from the regulatory systems which control their proliferation appears to be a good index of tumor progression.
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PMID:Cell proliferation kinetics in human solid tumors: relation to probability of metastatic dissemination and long-term survival. 266 9

Neuroblastomas represent a spectrum of diseases categorized by histological subtypes, age of the patient, and extent of tumor (stage) at diagnosis. In this study we analyzed Ha-ras p21 (protein with molecular weight of 21,000) expression immunohistochemically on 47 primary human neuroblastomas resected at diagnosis. The data demonstrate that the amount of the Ha-ras product correlates with a favorable prognosis (P less than 0.001) and early stages of disease at diagnosis (P less than 0.05). These findings from unmanipulated human neuroblastomas indicate that the Ha-ras gene product (p21) might play a role in the mechanism(s) controlling aggressiveness in this type of tumor in vivo and that the Ha-ras p21 detected by a simple and reproducible immunohistochemical procedure may be of clinical importance in predicting prognosis in patients with this malignancy.
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PMID:Expression of Ha-ras oncogene products in human neuroblastomas and the significant correlation with a patient's prognosis. 327 97

A case of trabecular carcinoma of the skin that resulted in prequarter amputation of the arm for local tumor aggressiveness is presented. The tumor was originally diagnosed as metastatic, most probably neuroblastoma, leading to inadequate local resection that resulted in recurrence with extensive regional nodal metastases. The importance of recognizing trabecular carcinoma as a primary tumor of the skin with a potential for recurrence and metastasis is underscored, primarily since initial total excision with adequate margins of resection has proven in most instances to be curative.
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PMID:Trabecular carcinoma of the skin simulating metastatic disease. 372 97

Nine human neuroblastoma were serially transplanted in nude mice. These tumor lines were established from the patients older than 1 year 10 month year old. None of the tumors taken from under the age of 1 year and 7 months grew in nude mice. All transplanted tumors produced catecholamines in varying degree. The growth of low catecholamines producing tumors was apparently aggressive compared with high catecholamines producing tumor lines. These results may indicate that tumor aggressiveness decreases with increased functional differentiation in human neuroblastoma in nude mice. The contents of catecholamine was closely correlated with the number and size of secretory granules in tumor cells. This human neuroblastoma/nude mice system may prove to be a useful tool for basic research on the biological behavior as well as on the cytological and functional differentiation of human neuroblastoma.
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PMID:Growth and functional behavior neuroblastomas in man and nude mice. 610 Jan 44

CD44 gene products are potential markers of aggressiveness in different tumour models, a result which prompted us to study clinical neuroblastoma (NB) specimens. CD44 expression was determined by immunostaining of 52 tumour samples from newly diagnosed NB with a monoclonal antibody (J173) directed against an epitope common to all CD44 isoforms. CD44 immunoreactivity was detected in 37 of the tumours (71%). CD44 was expressed in all 22 NBs with favourable prognoses (stages 1, 2 or 4S), but only 50% (15/30) of advanced NB (stages 3 and 4) (P < 10(-4)), suggesting that the absence, rather than the overexpression, of CD44 is a signal of tumour aggressiveness. The cumulative progression-free survival was significantly longer in patients with CD44 positive tumours compared with patients with CD44 negative tumours (P < 10(-5)). More importantly, progression-free survival was also significantly higher in CD44 positive patients within the high-risk group (P < 0.01). In univariate analysis, we tested the prognostic value of tumour expression of CD44 in comparison with tumour stage, age, tumour histology, and presence or absence of amplification of the MYCN protooncogene. All five measures had significant prognostic value. The expression of CD44 and the absence of MYCN amplification were the most powerful predictors of a favourable outcome. In a multivariate analysis of these measures, CD44 expression and tumour stage were the only independent prognostic factors for the prediction of patient survival. NB is the first clinical model described in which tumour aggressiveness correlates with repression rather than stimulation of CD44 expression. We recommend the use of CD44 as an additional biological marker in the initial staging of NB.
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PMID:Evaluation of CD44 prognostic value in neuroblastoma: comparison with the other prognostic factors. 757 64

More aggressiveness in treatment of childhood malignancies has had an evident impact on survival and rate of cure but, it has also allowed us to discover long-term effects of these treatments, and second malignant tumors of them. Between 1970 and 1993, 472 cases of malignant tumors in childhood were diagnosed in our department. Six of them (1.27%) developed a second tumor (five malignant and one benign). Relationship between first and second tumors are: seven years old boy, cervical lymphosarcoma-thyroid carcinoma; eleven years old boy, osteogenic sarcoma-vesical carcinoma: two years and six months old boy, cerebellar astrocytoma-soft tissue osteogenic sarcoma; five years old girl. Wilm's tumor-scapular osteogenic chondroma; one year and a half old girl, abdominal neuroblastoma-granulocytic sarcoma (chloroma); twelve years old boy. Hodgkin's disease-acute myeloblastic leukemia. All of them were clearly related to concogenic effect of radiation or chemotherapy.
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PMID:[Second tumors in childhood]. 776 70


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