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)

Delayed primary operation has been started since 1982 in our department and 19 cases of solid tumor in childhood were treated with this method. They included 11 cases of advanced neuroblastoma, 5 cases of hepatoblastoma and 3 cases of yolk sac tumor in sacrococcygeal area. We discussed effectiveness of preoperative chemotherapy to the primary tumor and metastatic lesions, the best timing of operation after chemotherapy and the operative technique in delayed primary operation. Preoperative chemotherapy were useful in almost all cases of delayed primary operation, so the primary lesion and lymph nodes metastases were removed easily. We recommended it was the best timing of operation when 2 or 3 courses of preoperative chemotherapy were finished. CUSA (Cavitron Ultrasonic Surgical Aspirator) was the useful instrument to remove the primary tumor and lymph node metastases completely in advanced neuroblastoma preserving both kidneys.
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PMID:[Delayed primary operation of solid tumors in children]. 284 15

The optimal management for patients with stage II neuroblastoma has not yet been established. In order to determine the impact of adding chemotherapy and/or radiation therapy to surgery, we reviewed by questionnaire 156 patients with stage II neuroblastoma treated by 28 Childrens Cancer Study Group (CCSG) institutions from 1978 to 1985. Survival and progression-free survival (PFS) were analyzed by life-table methods with respect to age at diagnosis, site and size of primary tumor, spinal cord involvement, extent of initial resection, and treatment in addition to surgery. The overall 5-year survival was 96%; the PFS was 90%, similar to previous CCSG studies. Age at diagnosis had a small impact on PFS, with 92% PFS for patients less than 2 years of age at diagnosis, and 84% for those greater than 2 (P = .10). The only site with an adverse outcome was the head and neck (n = 11), with a PFS of 68% compared with 93% for the remaining sites (P = .02). Size of primary and intraspinal extension of primary did not affect PFS. The extent of resection and subsequent treatment with radiation therapy and/or chemotherapy did not affect the PFS. The outcome for 75 patients treated with surgery alone (6-year PFS, 89%) was not significantly different from that of 66 patients receiving radiation therapy (6-year PFS, 94%). There was no significant difference between 40 patients with gross or microscopic residual disease treated with surgery alone (PFS, 92%) and 59 patients with residual disease who also received radiation (PFS, 90%). Five of seven patients who progressed after surgery alone have been salvaged with further therapy and are now free of disease. One survives with disease, so that the 6-year survival is 98% for those treated initially with surgery alone, compared with 95% for those receiving radiation therapy and/or chemotherapy. These data suggest that surgery alone, even if complete resection is not achieved, is sufficient initial therapy for stage II neuroblastoma. The data also identify another stage of neuroblastoma, in addition to stage IV-S, for which almost all patients have a favorable prognosis because their tumor may be biologically limited in growth.
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PMID:Excellent outcome of stage II neuroblastoma is independent of residual disease and radiation therapy. 1056 31

Human neuroblastoma is an immunogenic tumor for which therapy directed in an immunologic context may offer some advantage over conventional treatment. This study examines the immunomodulatory effects of surgery and irradiation in the murine C1300 neuroblastoma model. In vivo studies of primary tumor growth characteristics after treatment demonstrated no superiority of either therapeutic modality in control of local tumor or prolongation of host survival. However, irradiated hosts showed an increased ability to reject a secondary tumor challenge, compared to their surgical counterparts. That this phenomenon may be immune-related is suggested by in vitro studies of T lymphocyte function utilizing mixed lymphocyte-tumor cell cultures and PHA lymphoblastogenesis.
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PMID:Selection of optimal therapy for neuroblastoma: a study of the immunomodulatory effects of surgery and irradiation in the murine C1300 neuroblastoma model. 294 17

Collagens are a heterogeneous family of structural proteins synthesized by many cultured cells including tumor cells. The synthesis of these proteins by three human tumor types commonly encountered in children [neuroblastoma, rhabdomyosarcoma, and nephroblastoma (Wilms' tumor)] was investigated in short-term cultures of freshly excised tumor explants grown on extracellular matrices. Analysis of the incorporation of [3H]proline into collagenase-sensitive proteins indicated significant collagen production by several Wilms' tumors and rhabdomyosarcomas, while neuroblastomas did not synthesize this structural protein. All eight Wilms' tumor specimens analyzed secreted type IV procollagen. Interstitial types I and III collagens were also produced by these tumors, but in most cases, the alpha 1 (I): alpha 2 ratio was much higher than the 2:1 ratio expected for type I collagen, indicating a major change in the control of type I collagen production. Rhabdomyosarcomas were very heterogeneous with regard to collagen secretion and synthesized either a single collagen isotype (type III), several collagens including types I, III, and IV, or no detectable collagen. Our data represent a first quantitative and qualitative analysis of collagen synthesis by primary tumor cultures and reveal much more heterogeneity in collagen biosynthesis by these tumors than reported previously with established cell lines. They also indicate significant alterations in the expression of type I collagen genes in Wilms' tumors.
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PMID:Collagen synthesis by short-term explants of pediatric tumors. 298 85

We reviewed the autopsy, clinical, and radiographic records of 39 patients with metastatic skeletal disease (age range 18 months-20 years). There were 11 different primary tumors responsible for skeletal metastases, of which neuroblastoma was the most common (16 patients). Other commonly occurring tumors giving rise to secondary skeletal deposits were rhabdomyosarcoma (seven), teratoma-teratocarcinoma (four), and Wilms tumor (three). Overall patient survival ranged from 2 weeks to 72 months, with an average of 10.2 months. Those patients presenting without skeletal metastases at the time of diagnosis of the primary tumor had a survival time averaging 16.2 months (range 3-72 months), whereas those patients presenting initially with skeletal metastases at the time of diagnosis of the primary tumor had a survival time of 8.5 months (range 0.5-23 months).
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PMID:Metastatic skeletal disease in the pediatric population. 298 4

Skeletal involvement in childhood nonosseous tumors can be due to primary involvement, arrosion or metastasis, secondary due to therapy induced alterations or osteomyelitis following diminished immunity. The occurence of bone changes differs widely from those in adults. Neuroblastoma, rhabdomyosarcoma and malignant lymphoma are discussed in detail. Rare tumors are listed for synopsis. As diagnostic screening method skeletal scintigraphy is recommended, whereas in localized disease X-rays should be performed. Beside roentgenmorphology-particularly in primary disease-localisation, frequency and age dependency may give essential diagnostic hints. Prognosis depends on primary tumor.
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PMID:[Skeletal manifestations of malignant, nonosseous tumors in childhood]. 298 8

A two-step evaluation system is proposed to analyze the results of therapy for stage IV neuroblastoma in children over 1 year of age. Since most protocols consist of first-line chemotherapy followed by surgery and often high-dose chemotherapy with bone marrow rescue, "response" should assess the effect of chemotherapy alone and "remission" indicate the status after an attempt to remove the primary tumor. A scoring system is proposed for both steps. The comparability of different studies depends on the use of such common criteria to define patient groups and treatment results.
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PMID:Definition of response and remission in children over one year of age with advanced neuroblastoma: proposition for a scoring system. 315 10

Fourteen scans employing the adrenergic blocking agent [131I]MIBG were performed on 10 children with neuroblastoma (NB) or ganglioneuroblastoma (GNB). The scans were negative in 5 cases, and 1 further case produced doubtful results in both the MIBG and CT scan tests. In 4 cases, very positive results were obtained with clear vision of the primary tumor and its metastases. In 1 case, which demonstrated partial differentiation of the outer part of the tumor mass toward GNB, a differentiated tumor specimen did not reveal significant uptake of the tracer. Half-lives of the tracer as measured by external detection in the period 24-48 h after injection were reduced after successful therapy. MIBG scanning appears to be a feasible indicator of NB adrenergic activity, and it can assume a primary role in the staging and follow-up of NB. Higher tumor uptake of the [131 I]MIBG and low background offer new perspectives in the radiometabolic treatment of MIBG.
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PMID:The role of meta-iodo [131I]benzylguanidine (MIBG) in the diagnosis and follow-up of neuroblastoma. 315 17

The data on 26 patients with solitary metastatic lesions arising in cortical bone were studied. Nineteen patients were over 50 years of age. In 19 patients, the cortical metastasis was the first indication of the presence of a primary malignant condition. In seven cases, cortical metastases developed in patients with a known primary tumor. The primary tumors involved were eight renal cell carcinomas, six bronchogenic carcinomas, two carcinomas of the gastrointestinal tract, one osteosarcoma, one neuroblastoma, one melanoma, one hepatoma, one carcinoma of the breast, and one thyroid carcinoma. In four cases, the primary tumor remained unknown. A metastatic origin should be considered in the differential diagnosis of an osteolytic lesion arising in the cortex of a long bone, especially in older patients and in patients with a known primary malignant condition. The cortical bone metastases encountered in this study did not originate solely from bronchogenic carcinoma, as has been reported by other authors. Cortical metastases are probably less rare than has been hitherto assumed.
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PMID:Cortical bone metastases. 317 2

A 18-month-old boy with stage 4 neuroblastoma needed intensive care because of prerenal acute renal failure related to an intractable watery diarrhoea syndrome occurring 10 months after the diagnosis of the primary tumor. This diarrhoea was in relation with a late hyperproduction of vasoactive intestinal peptide by the relapsing neuroblastoma itself and stopped with intravenous somatostatin administration.
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PMID:[Metastatic neuroblastoma with secondary hypersecretion of vasoactive intestinal peptide]. 320 Jun 52


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