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

Thirteen children with localized (Evans stage I or II) thoracic primary neuroblastoma were divided into two groups according to the type of therapy administered, in order to compare the therapeutic efficacy and morbidity of excisional surgery followed by either irradiation alone or irradiation plus chemotherapy (group A) with similar surgery alone (group B). Group A consisted of 6 children (mean age 1 year, 2 months). Complete surgical excision was accomplished in 2 patients, while 4 had microscopic residual. All 6 patients are free of disease at 26--76 months (mean 47 months), including 2 who had recurrent tumor and received additional therapy. Two have developed congestive heart failure and one severe scoliosis secondary to irradiation. Of the 7 children in group B (mean age 2 years, 2 months), 3 had microscopic residual tumor and 2 had adjacent lymph node involvement. After 12--47 months (mean 23 months), no recurrence or surgery-related morbidity has been observed. From these limited data it appears that surgery alone may provide adequate therapy for localized thoracic neuroblastoma and obviate the morbidity associated with multimodal therapy.
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PMID:Management of localized thoracic neuroblastoma. 50 72

Neuroblastomas are malignant childhood neoplasms that arise from derivatives of the neural crest. We report the characterization of a new neuroblastoma cell line, designated NBL-W, derived from the primary tumor of a patient with stage IVS disease (S. L. Cohn, C. V. Herst, H. S. Maurer, and S. T. Rosen, J. Clin. Oncol., 5: 1441-1444, 1987) according to the criteria of Evans [A. E. Evans, G. J. D'Angio, and J. Randolf, Cancer (Phila.), 27: 374-378, 1971]. Neurite-bearing (N) and substrate-adherent (S) cell lines have been subcloned from the parent line. N and S cells can interconvert, and both cell types label with the neural crest cell surface marker antibody, HNK-1. Cells in the subcloned lines and in the parent line have been shown by Southern blot analysis to contain approximately 100 copies of the N-myc gene. Cytogenetic analysis shows a homogeneously staining region present on chromosome 19. Although these subclones are of identical genotype, the S cells express lower amounts of N-myc mRNA and protein as compared to the N cells. N cells express several neuronal proteins including the neurotransmitter-processing enzymes tyrosine hydroxylase and dopamine beta-hydroxylase, the neuronal intermediate filament proteins peripherin and NF66/alpha-internexin, and the neural cell adhesion molecule. S cells generally lack neuronal markers but express the mesenchymal intermediate filament protein vimentin, and a small subset of the S cells express glial fibrillary acidic protein. Some S cells were labeled weakly with neural cell adhesion molecule antibody; others were negative. S cells did not express the glial marker S-100 or a melanocyte marker, tyrosinase. Thus, S cells express the neural crest marker HNK-1 but do not express a set of antigens characteristic of any known cell type derived from the neural crest. These results are consistent with the suggestion that differential N-myc expression may be involved in the interconversion of N and S cells but indicate that the S cell phenotype need not represent a highly differentiated neural crest derivative.
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PMID:Differential expression of N-myc in phenotypically distinct subclones of a human neuroblastoma cell line. 193 96

From 1984 to 1989, 47 children with relapsed, refractory, and/or metastasized neuroblastoma were treated with 131I-metaiodobenzylguanidine (mIBG) in several different treatment combinations. At initial diagnosis, 36 children had Evans stage IV and 11 stage III disease. In 16 of the 47 children, tumor recurred after complete remission prior to mIBG treatment, 26 of 47 progressed from residual or nonresponding tumor, and in 5 of 47 tumor progression during chemotherapy was observed. Altogether the children were treated with a total of 112 courses (range 1-6) with a mean dosage of 8.9 +/- 6.7 mCi/kg body weight/treatment course. Total dose was 283.2 +/- 203.7 mCi for stage III and 388.9 +/- 218.6 mCi for stage IV. Nine of 47 children reached a complete or a very good partial remission (CR and VGPR) from mIBG treatment alone, 13 of 47 achieved partial remission (PR). In an early analysis, 10 patients treated with mIBG in the neuroblastoma trial NB 85 of the German Society of Pediatric Oncology showed no significant difference in survival time compared with 30 conventionally treated children. However, the recent therapy series has been done with higher doses of mIBG, and during improved therapeutic scanning many more bone lesions could be detected than during earlier diagnostic scanning. We conclude that mIBG treatment has not yet fulfilled the expectations for it but still seems for certain indications to be a promising tool to treat neuroblastoma in the future. Moreover, the frontier of neuroblastoma detection is still advancing.
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PMID:Metaiodobenzylguanidine (mIBG) in treatment of 47 patients with neuroblastoma: results of the German Neuroblastoma Trial. 201 Nov

A new human neuroblastoma cell line (LS) that originated from an abdominal tumor of a 16-month-old girl is presented; it was classified, according to Evans, as being stage III. Morphological (dense-core particles) and biochemical characteristics (dopamine-beta-hydroxylase, acetylcholinesterase, neuron-specific-enolase) confirmed the diagnosis. In addition to a slightly variable modal chromosome number of 48 or 49 (because of marker-chromosomes and autosomal trisomies), cytogenetic analysis revealed two constantly appearing chromosomes with homogeneously stained regions (HSR's). The karyotype remained constant over 50 passages in vitro [49,XX, -12, +der5, + 17, + mar1, + mar2]. Double minutes were a rare phenomenon and appeared only in a few metaphases. In situ hybridization showed that some of the HSR's consisted of amplified N-myc copies. The distribution of the N-myc copies according to in situ hybridization signals along the HSR's was compared with the data of Southern and Northern blotting analyses.
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PMID:Cytogenetic and molecular characterization of a newly established neuroblastoma cell line LS. 202 21

The objectives of this study were to determine (1) the role of selection before bone marrow transplantation (BMT), (2) the role of vincristine, melphalan, and total body irradiation (TBI) as consolidation of induction therapy for stage IV over 12 months at diagnosis, and (3) the role of immunomagnetic purging in metastatic neuroblastoma. Among 72 consecutive unselected patients, 10 were not grafted (four died at induction: two in complete remission [CR], two in partial remission [PR]); three had bone marrow progression before harvest; one had uncontrolled progression; and two had parental refusal). Sixty-two patients were grafted (23 in CR/very good PR [VGPR] and 39 in PR). Among the 62, 33 were consolidated with at least 90% excision of their initial tumor excised (53.2%), 15 with catecholamine secretions (24.2%), 22 with minor bone marrow involvement (35.5%), and 31 with positive bone scan (50%). Median observation time is 59 months. Progression-free survival (PFS) for the 10 excluded patients was 20% at 2 years and 0% at 4 years. PFS for the grafted population (n = 62) is 40% at 2 years, 20% at 4 years, and 13% at 7 years. No difference was observed between patients grafted in CR/VGPR or in PR. However, a group of 19 children was grafted resulting in complete normalization of metastasis (regardless of primary-site tumor status). In this group, PFS at 59 months was 38% with no relapses up to 7 years post-BMT. A group of 31 patients with no bone involvement at BMT was also identified. PFS at 5 years is 30% compared with 12% for bone-positive patients at BMT. Moreover, the 11 children presenting at diagnosis with no bone involvement (Evans stage IVS or stage C Memphis) and consolidated with BMT had PFS at 5 years of 50% with no late relapses. A subgroup of stage IV neuroblastoma patients older than 1 year of age at diagnosis may be curable with this therapeutic approach, and the use of multivariate analyses to search for prognostic factors is warranted in currently existing international registries.
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PMID:Improved survival at 2 and 5 years in the LMCE1 unselected group of 72 children with stage IV neuroblastoma older than 1 year of age at diagnosis: is cure possible in a small subgroup? 203 17

In 1971, the Japanese Society of Pediatric Surgeons' Committee on Malignancies proposed new criteria for neuroblastoma staging. It was fundamentally, based on the system of Evans et al. described in 1971. The main difference was the separation of stage IV disease into stages IV-A, with metastases to bone, orbita, distant lymph nodes and viscera other than liver, IV-B, the primary tumor extending over the midline and with metastases to bone marrow, liver and skin, and IV-S, which was the same as that of Evans et al. The new criteria did not include the resectability of the primary tumor, assessment of regional lymph node involvement or any other disease assessment resulting from therapeutic intervention. For the purpose of international usage, the Japanese system has been newly formulated and proposed as the Japanese Tumor Node Metastasis (TNM) Postsurgical Histopathological Classification for Neuroblastoma. In the present report, 495 neuroblastomas, registered between 1970 and 1985, were analyzed retrospectively according to the International Union Against Cancer (UICC) TNM classification and the proposed Japanese TNM system. The analyses suggested that the Japanese system reflected both the extent of tumor invasion and its biological neuroblastoma characteristics better than the UICC TNM classification based on statistical analysis.
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PMID:Proposal and assessment of Japanese tumor node metastasis postsurgical histopathological staging system for neuroblastoma based on an analysis of 495 cases. 206 17

Our policy of preoperative and postoperative adjuvant therapy of advanced neuroblastoma changed from mild to aggressive chemotherapy in 1978. In order to evaluate the clinical effects of this policy, 39 cases before 1977 and 37 cases after 1978 were retrospectively reviewed using the Evans and the International Union Against Cancer (UICC) staging systems. Both clinical stages (CS) and postsurgical histopathological stages (PS) of the UICC staging system showed an almost 100% cure rate in stages I (CSI, PSI) and II (CSII, PSII). The cure rates of CS III, PS IIIA, and PS IIIB cases were remarkably improved after 1978, but those of CS IV and PS IV cases remained extremely low. However, it was found that a disease-free survival rate of advanced neuroblastoma could be produced by complete resection of the tumor accompanied by adjuvant chemotherapy. In these conditions, we found that for predicting the prognosis of advanced neuroblastoma, the UICC staging system, especially the PS staging system, may be more rational than the Evans staging system.
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PMID:Fifteen years' experience of neuroblastoma: a prognostic evaluation according to the Evans and UICC staging systems. 231 3

We have evaluated the role of radiotherapy in providing local control of primary tumors and to palliate metastases from neuroblastoma (NB). Fifty-five children with histologically verified NB were evaluated and treated from 1967 to 1984. In univariate analysis, the actuarial survival of eight children with thoracic primaries (85%) was significantly better than the survival of 39 children with intra-abdominal primaries (35%, p = 0.0287). The survival of 28 children less than or equal to 18 months of age at diagnoses was 73%, whereas 27 children older than 18 months had a survival probability of 10% (p = 0.0001). The survival by Evans stage was: I 100% (2 patients), II 85% (7), III 60% (13), IV 4% (27) and IV-S 100% (6). According to the Pediatric Oncology Group (POG) staging system, the survival was: A 100% (3), B 66% (9), C 66% (9), D 23% (34). A multivariable analysis indicated that the Evans staging system was a more powerful indicator of prognosis than the POG system. The analysis also indicated that Evans stage and patient age were independent determinants of survival. The primary tumor site did not add significant prognostic information beyond these two factors. Children with Stage I disease were treated with surgery alone. Most children with Stages II and III disease were treated with surgery, irradiation, and Cyclophosphamide or Cyclophosphamide plus Vincristine. All seven patients with Stage II disease received post-operative irradiation to the primary tumor and were locally controlled with doses of 4.8 to 26.5 Gy. Eleven of the 13 patients with Stage III disease were irradiated post-operatively. Seven of these 11 patients were locally controlled with doses of 12 to 48.4 Gy. The four Stage III patients with in-field recurrences were older children with large radiotherapy fields and/or low doses administered. The Radiation Therapy Oncology Group pain score system was used to evaluate response of painful bony metastases to irradiation. A response was observed in 65% of the sites irradiated. A response was observed at 67% of the soft tissue metastases irradiated. Hepatomegaly causing respiratory embarrassment or inferior vena cava obstruction was treated with irradiation in seven patients. All patients responded with doses ranging from 5 to 24.4 Gy. Five of the 17 children who survived for more than 5 years following treatment had significant scoliosis or kyphosis secondary to vertebral body abnormalities in irradiated bones. All five children were irradiated at a young age with megavoltage equipment.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Radiation therapy in the management of neuroblastoma: the Duke University Medical Center experience 1967-1984. 242 88

In the three most widely used neuroblastoma staging systems--Evans, Pediatric Oncology Group (POG), and TNM-Union Internationale Contre le Cancer (UICC)--few staging problems are presented by the completely resected tumor or with the patient who has metastatic disease. Difficulties arise with the localized tumor when there is extension across the midline and with the demonstration of lymph node involvement. A new International Staging System for Neuroblastoma (INSS) is proposed that incorporates both lymph node invasion and extension across the midline into surgical staging. Clear criteria for diagnosis are established and clinical responses are defined. New prognostic factors may be added. The surgical role remains significant in staging, diagnosis, and therapy. Use of a common system throughout the world would facilitate the evaluation of clinical trials and help achieve the goal of developing optimum therapy for the biologically different types of neuroblastoma.
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PMID:A surgical perspective on the current staging in neuroblastoma--the International Neuroblastoma Staging System proposal. 273 83

Human neuroblastoma (NB) is a highly malignant tumor arising in cells that originate in the embryonal neural crest. Several lines of investigation suggest that both NB and other tumors of developing tissues are blocked in their ability to differentiate and achieve growth arrest. Since in vivo differentiation of NB has been frequently observed and may be of clinical importance (Fox et al., 1959; Evans et al., 1976), we have utilized the in vitro induction of NB differentiation by retinoic acid (RA) to study the molecular events associated with NB differentiation. We have focused our studies on changes that occur in the expression of various proto-oncogenes during NB tumors cell differentiation because proto-oncogenes are likely to be of central importance in mediating processes critical for cellular growth and maturation. In these studies, we have found that the expression of no fewer than five proto-oncogenes including c-Ha-ras, c-ets-1, and c-fos change during the differentiation of NB cells, while the expression of c-erb-B changes in association with the arrest of growth that occurs during NB differentiation. In some cases the altered expression of a proto-oncogene was transcriptionally regulated, while in others post-transcriptional mechanisms were important.
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PMID:The expression of multiple proto-oncogenes is differentially regulated during retinoic acid induced maturation of human neuroblastoma cell lines. 306 Jul 92


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