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)

In 1973, mass screening program for 6-month old infants for early detection of neuroblastoma using a VMA spot test of a urine sample was initiated in Kyoto. In 1985, nation wide mass screening was initiated throughout the entire country and the Government has given the financial support to each district. In 1988, the Government recommended the institution of mass screening by quantitative measurements of VMA, HVA and creatinine using HPLC (high performance liquid chromatography), instead of the qualitative test of VMA alone. From 1974, at the time of initiation of mass screening for neuroblastoma to the end of October, 1989, 383 cases with this tumor have been discovered throughout the screening program. Three hundreds eighty three cases (88%) of them had been registered to the Neuroblastoma Committee of the Japanese Society of Pediatric Oncology. In this paper, the mass screening program was introduced and the 337 cases with this tumor detected by 6-month old screening were analyzed their clinical symptoms, findings, urinary VMA and HVA levels, primary sites, weights of primary tumor, histology, stages at diagnosis, metastatic sites, and the results of the treatment. Three hundreds twenty eight cases (97%) of them are expected to be cured. And we discussed clinical problems related to mass screening program for neuroblastoma, such as an increase of the incidence of infantile neuroblastomas detected by this program and the spontaneous regression.
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PMID:[Present status of neuroblastoma mass screening in Japan. Neuroblastoma-Committee of the Japanese Childhood Cancer Society]. 226 Aug 68

Genomic amplification of the oncogene N-myc is associated with rapid tumor progression and poor prognosis in patients with neuroblastoma (NB). However, 40% of NBs which lack N-myc amplification are also clinically aggressive. Factors other than N-myc copy number must therefore play a role in determining tumor progression in these NBs. We have established an unusual human NB cell line (NBL-S) from the primary tumor of a patient with rapidly progressive disease which lacks N-myc amplification. The doubling time in vitro (48 h) and the time from injection of 2 x 10(7) cells to detectable tumors in nude mice (46 days) in similar to NB cell lines with amplified N-myc. However, karyotype analysis reveals no evidence of double minutes (DMs), homogeneously staining regions (HSRs), or chromosome 1p deletions, features commonly seen in NB cell lines. The cells have the cell surface phenotype typical of N-myc amplified NB (HLA-A,B,C negative and HSAN 1.2 positive), and similar to other NB cell lines, N-myc RNA and protein are expressed. Interestingly, the half-life of the N-myc protein in NBL-S is prolonged (approximately 100 min) compared to the short N-myc protein half-life previously described in N-myc amplified NB cell lines (approximately 30 min). Because N-myc protein is thought to have a regulatory role, prolongation of the half-life of this protein may be an important factor in the regulation of growth in NBs which lack N-myc amplification and rapidly progress.
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PMID:Prolonged N-myc protein half-life in a neuroblastoma cell line lacking N-myc amplification. 228 1

Four major staging systems have been used to estimate the prognosis for children with local and regional neuroblastoma (NBL). Data obtained at diagnosis for 251 neuroblastoma patients from two Childrens Cancer Study Group (CCSG) studies were analyzed according to staging systems of the CCSG, St Jude Children's Research Hospital, the Pediatric Oncology Group (POG), and the Union Internationale Contre le Cancer (UICC) tumor-nodes-metastasis (TNM) system. The most significant variables were found to be age, tumor stage, extent of tumor removal, transgression of the midline by tumor infiltration, and site of primary tumor. Involvement of lymph nodes per se was not a bad prognostic sign unless associated with extension beyond the midline, the latter being the single most important prognostic variable. All four staging systems had value for prognostication and all identified with accuracy the low stage patient (stage I, stage A) who fares well (greater than or equal to 87% survival). The CCSG definition of stages II and III disease discriminated prognostic groups best among the remaining patients, and was able to identify the child with local-regional NBL with poor survival. The estimated 5-year survival rates for children with regional tumor (stage III, IIIA[N]), according to the four systems were 44%, 74%, 74%, and 74% for the CCSG, St Jude, POG, and UICC methods, respectively. We conclude that all four staging systems effectively define good-prognosis patients with localized disease but that the CCSG staging system most accurately identifies patients with regional tumor who have a poor outcome.
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PMID:A comparison of four staging systems for localized and regional neuroblastoma: a report from the Childrens Cancer Study Group. 198 68

We have examined two features of neuroblastoma cells that had not been well-characterized in a xenogeneic model: The cells display unusual immunologic properties in other experimental systems, and the original tumors display widespread and characteristic patterns of metastasis. To determine the most appropriate immunodeficient host for primary tumor growth, T cell-deficient nude mice, NK-deficient beige mice, beige-nudes, and controls were injected with the well-characterized line CHP-100. To define the pattern of tumor spread, complete autopsies were performed following subcutaneous, intraperitoneal and intravenous injections. CHP-100 consistently formed subcutaneous tumors in T cell-deficient mice (nude and beige-nude), but not in T cell-competent mice (beige, heterozygous nu/+ and bg/+, or wild-type). The growth rate and final size of the subcutaneous tumors were not greater in beige-nudes than in nudes. All mice showed early CHP-100 cell death after subcutaneous injection; the nature of the immunodeficiency was more relevant for the surviving subpopulation. Widespread dissemination was seen following intravenous injection, particularly in beige-nudes. Aspects of the growth patterns were appropriate to the tumor of origin. The behavior in immunodeficient mice suggests that T cells can play a role in controlling the growth of these cells; the next steps will be to define the effector mechanisms, and to determine if they can be exploited for human patients. The hematogenous spread following intravenous injection suggests that insights into the control of blood-borne tumor may also come from further study of this model.
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PMID:Human neuroblastoma cell growth in xenogeneic hosts: comparison of T cell-deficient and NK-deficient hosts, and subcutaneous or intravenous injection routes. 235 46

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

The significance and indications of MIBG scintigraphy are critically assessed. The results are compared with the results of whole-body bone scintigraphy, computed tomography (CT) and magnetic resonance tomography (MRT), and are related to values of catecholamine metabolites in 24-h urines. In our patients (10 histologically proven cases) MIBG scintigraphy turned out to be most useful in tumor follow-up. In contrast, the significance was much lower in primary tumor diagnosis and tumor staging as the exact primary diagnosis was established by other means such as CT, MRT, MDP whole-body scan, urine chemistry and bone marrow biopsy in all cases. MIBG scintigraphy in diagnostic imaging of neuroblastoma is an additive diagnostic tool and is called for in (1) tumour follow-up (progress, recurrencies, metastases); (2) primary diagnosis if the primary tumour has not been localized by means of CT or MRT; and (3) tumour staging to differentiate stage IV disease from lower stages as long as stage IV disease has not been established by bone-marrow biopsy or MDP whole-body scan.
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PMID:[Indications for MIBG scintigraphy in the diagnosis of neuroblastoma]. 271 Jun 44

Fifty-eight patients with stage III neuroblastoma (according to the Childrens Cancer Study Group) underwent 86 operative procedures. Initial diagnostic or therapeutic operations were followed by chemotherapy and radiation therapy, and second- or third-look procedures were performed on patients whose tumors showed evidence of clinical response. Complete resection was obtained during the initial procedure in 12 patients, during the second operation in 12 others, and at the time of the third procedure in two additional children. Twenty of these 26 patients have survived with no evidence of disease for a median duration of greater than 3 years since initiation of therapy. Only nine of the 32 patients in whom complete tumor excision could not be accomplished are still alive. The chemotherapy and radiation therapy employed in this protocol may have enhanced resectability in many patients. The site of the primary tumor does not seem to influence ease of resectability or outcome in this group. The "favorable" prognostic factors of patient age and histopathology of the tumor do not necessarily predict that the tumor will be resectable. While patients with complete resection at the time of the second operation had an outcome superior to those with incomplete resections, the influence of the inherent tumor biology or other selection factors on the eventual tumor resectability in these patients is not known. Extensive operative procedures were required to completely resect stage III tumors, and a moderate complication rate may be anticipated. However, this may be justifiable if complete tumor resection can be achieved and ultimate patient survival possibly enhanced.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Improvement in survival after excision of primary tumor in stage III neuroblastoma. 272 13

Fourteen children with histopathologically confirmed neuroblastoma underwent sequential correlative imaging studies using I-131 MIBG, Tc-99m MDP, and Ga-67 citrate during various stages of the disease. Of the patients 86% showed I-131 MIBG accumulation in the primary tumoral site, whereas 71% showed Tc-99m MDP and 79% Ga-67 citrate uptake. In 86% at least one of the two latter radiopharmaceuticals concentrated in the primary tumor. The use of all three radiopharmaceuticals raised the detection rate to 93%. Of the osseous or extraosseous metastases 100% were detected by Tc-99m MDP studies. The I-131 MIBG studies were positive in 71% of the osseous metastases and in 70% of the extraosseous metastases. No Ga-67 citrate uptake was demonstrated in osseous metastases, although one extraosseous lung metastasis concentrated this radiopharmaceutical. Tc-99m MDP bone imaging was the best method for diagnosing metastatic spread of the disease and for monitoring the results of treatment. Primary tumor uptake was best indicated by I-131 MIBG. Both Ga-67 citrate and I-131 MIBG were superior to Tc-99m MDP with regard to accurately demonstrating the extent of primary tumors. Only Tc-99m MDP indicated the relationship of these tumors to the kidneys and neighboring osseous structures, providing early screening of kidney compression. Ga-67 citrate study was mainly indicated in tumors with catecholamine depletion, which failed to concentrate the other two radiopharmaceuticals. I-131 MIBG proved especially useful in detecting neuroblastoma with negative Tc-99m MDP and Ga-67 citrate studies and also proved to be helpful with those cases in which I-131 MIBG was planned for therapy. The following strategy is suggested for evaluating neuroblastoma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Neuroblastoma: imaging evaluation by sequential Tc-99m MDP, I-131 MIBG, and Ga-67 citrate studies. 276 33

A prerequisite for the study of biological characteristics in neuroblastoma is the establishment of cell lines from tumors of patients. For our study a neuroblastoma cell line, IGR-N-835, was established from a primary tumor. During in vitro establishment of this line morphological changes were observed. IGR-N-835 exhibited both anchored cells and floating clusters. When cultured on bovine endothelial corneal cellular matrix, all tumor cells anchored to the matrix and proliferated, giving a continuous cell line. IGR-N-835 was studied in vitro and in vivo. Treatment with retinoic acid resulted in cell growth arrest and morphological differentiation. IGR-N-835 was highly tumorigenic in nude mice, exhibited a doubling time of 65 hr and was able to form colonies in methyl-cellulose with a cloning efficiency of 0.46%. Immunocytochemical studies showed reactivity of this line and its primary passages with CE7 monoclonal antibody (MAb). Cytogenetic analyses revealed stable mode and markers resulting from structural changes in chromosomes 10, 11 and 21 with no homogeneously staining regions or double minute chromosomes. Genomic amplification and overexpression of the N-myc oncogene exhibited by the patient's tumor remained unchanged in nude mouse xenografts and the IGR-N-835 cell line. These phenotypic and genotypic traits were unchanged during establishment of this neuroblastoma line. IGR-N-835 could therefore constitute a suitable material for the study of the biology or therapeutics of human neuroblastoma.
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PMID:Stability of phenotypic and genotypic traits during the establishment of a human neuroblastoma cell line, IGR-N-835. 277 12

Twenty neuroblastoma and 4 nonneuroblastoma patients were studied by 131I-MIBG imaging. The primary tumor was detected in 89% of patients (8/9) before therapy. Bone marrow metastasis was also visualized in 4 of the 8 patients with primary positive scan. True negative results were obtained in 4 nonneuroblastoma patients. After therapy, of 10 tumor-bearing patients, eight showed positive scans and 9 of 12 lesions (75%) were visualized. The accuracies of presence or absence of neuroblastoma were compared between 131I-MIBG imaging and several tumor markers. The accuracies before and after therapy were as follows: 131I-MIBG imaging; 92% (12/13), 88% (15/17), serum NSE; 80% (4/5), 93% (13/14), serum LDH; 92% (11/12), 76% (13/17), urinary VMA; 54% (7/13), 56% (9/16), and urinary HVA; 77% (10/13), 56% (9/16). It appears that 131I-MIBG imaging is useful for both locating and excluding neuroblastoma. In addition, 131I-MIBG imaging appears to be the most efficient diagnostic and follow up study for neuroblastoma when it is combined with measurements of serum NSE.
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PMID:[Clinical evaluation of I-131 metaiodobenzylguanidine (MIBG) imaging in suspected neuroblastoma]. 281 Sep 11


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