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)

Children with neuroblastoma treated in Salt Lake City from 1966 through 1982 were analyzed in an attempt to develop guidelines for external beam radiation. Particular attention was addressed to time-dose relationships in those patients with residual disease post-resection (Stages II and III). Altogether, 76 patients were analyzed and survival rates were: Stage I--100%; Stage II--84%; Stage III--69.2%; Stage IV--14.3%; Stage IV-S--71.4%. Survival rates were correspondingly better in younger children and in infants. Indications for postoperative radiation therapy in this population were: unresectable or gross remaining tumor; residual tumor in neural foramina; tumor spill during surgery; positive regional lymph nodes or positive surgical margins. Local control was achieved in a majority of patients undergoing surgery and radiation for limited disease. In children younger than 1 year of age, no local failures were observed at doses above 1200 rad. In children between 1-2 years of age, no local failures were observed with doses as low as 1440 rad. In children older than 3 years, local failures were observed up to 4500 rad.
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PMID:Dose response analysis of pediatric neuroblastoma to megavoltage radiation. 644 1

Four human neuroblastomas transplanted into nude mice were used for experimental chemotherapy and surgery, and the following results were obtained. Cyclophosphamide was the most effective for human neuroblastoma, cis-platinum being the second, among several chemotherapeutic drugs examined. Aclacinomycin A is more effective than Adriamycin. VM26 should be administered 48 to 72 hours after injection of cis-platinum, according to flow cytometric analysis. Flow cytometric analysis also disclosed that residual tumor grows most rapidly seven days after subtotal excision. However, chemotherapy is more effective in the postoperative period than it is in the preoperative period.
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PMID:Nude mouse xenograft study for treatment of neuroblastoma: effects of chemotherapeutic agents and surgery on tumor growth and cell kinetics. 658 77

Therapy was designed to achieve a high cure rate and to prevent serious therapeutic side effects for 11 infants younger than one year old with neuroblastoma who had a favorable prognosis (Evans Stages I, II, III, and IV-S). It consisted of surgery alone if the tumor was totally removed (one infant) and of surgery and low doses of cytoxan and vincristine for a period of 1 year if the tumor was incompletely removed (seven infants). In addition, radiation therapy was applied to unresected dumbbell tumors (three infants). All infants are alive without evidence of disease with the exception of one who died in an accident. The follow-up time varies from 2-8 years. The drug combination prevented recurrences in two infants whose tumor was reduced by surgery to less than 10% of the original size. In five infants, chemotherapy reduced the size of large residual tumor masses. Two of these masses were subsequently removed. The tumors of the three other infants recurred while on chemotherapy and were successfully eradicated by surgery or radiation therapy. Two infants were not treated according to this therapeutic plan. Although they had small residual masses after surgery, no chemotherapy was given. They are alive without recurrence of the disease 2 years or more after diagnosis. In summary, cure was achieved in these infants without intensive chemotherapy.
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PMID:Neuroblastoma: therapy for infants with good prognosis. 685 97

A group of 12 children with advanced neuroblastoma (7 Stage IV and 5 Stage III), selected by their initial response to chemotherapy with pulsed cyclophosphamide/vincristine/Adriamycin (CVA), were given consolidation therapy with high-dose melphalan (140 mg/m2) and then surgical removal of residual disease. Twenty-two high-dose melphalan procedures were combined with autologous marrow grafting to offset myelotoxicity and were well tolerated. In each of 2 additional children, procedures carried out without marrow autografting led to serious marrow and mucosal toxicity. There were no treatment-related deaths. In 7/11 patients with evaluable computerized tomographic (CT) scans there was a decrease in maximum diameter of the primary tumour after melphalan. Complete response was achieved in 6 patients, of whom 3 are well and have no evidence of disease at 35, 33 and 18 months from completion of all treatment; however, although survival (median 23 months) of all 12 autografted patients is longer than that of 28 comparable children treated between 1970-77 with conventional chemotherapy (median 14 months) the difference is not statistically significant. High-dose melphalan is a safe and tolerable treatment in children when combined with autologous marrow grafting, but further study is required to determine whether the procedure can improve prognosis for patients with advanced neuroblastoma.
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PMID:High-dose melphalan with autologous marrow for treatment of advanced neuroblastoma. 703 33

Five patients, 1.5 to 9 years old, were treated at M.D. Anderson Hospital for Stage III of IV neuroblastoma. Each was entered into a chemotherapy protocol which included cyclophosphamide, vincristine, 5-trifluoromethyl-2'-deoxyuridine (F3TdR), and papaverine, and each responded with marked reduction in size of the abdominal tumor mass. All patients then underwent exploratory laparotomy and resection or biopsy of residual tumor. In three cases, histological examination of the treated tumor showed ganglioneuroblastoma with predominance of mature cells; one patient had a mature ganglioneuroma, whereas only necrotic tissue was obtained from the fifth patient. These findings support the hypothesis that this chemotherapy protocol can induce neuroblastoma maturation.
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PMID:Induction of neuroblastoma maturation by a new chemotherapy protocol. 708 96

From January, 1969 through July, 1979, 37 children with neuroblastoma were cared for at the Oklahoma Children's Memorial Hospital. Nineteen patients with extensive disease were studied to examine the interrelationships of chemotherapy, radiation therapy and secondary surgery. Eleven children had secondary surgery in the abdomen or cervical region with one postoperative death. All patients had chemotherapy and ten had radiation therapy between the primary and secondary operation or death. Five children survived. Four of five survivors were less than one year of age at diagnosis and initial treatment. Fourteen of 15 patients, one year of age or older, died. Each case had pathologic examination of tumor before and after therapy. All survivors showed sequential maturation of tumor tissue but only one nonsurvivor had this finding. Unusual metastatic spread was found in patients having combined therapy. Multimodal therapy for advanced neuroblastoma accentuates the need for sensible timing and utilization of secondary operative procedures. The secondary procedures ideally eradicate the primary focus of tumor, and may also serve to excise selective areas of metastatic disease or to biopsy residual disease in aiding continuing therapy. Secondary surgery ought to follow the onset of radiation therapy by four to six weeks, or of chemotherapy by 12 to 24 weeks. This delay allows maximum control of primary and generalized disease, as well as maturation, differentiation, encapsulation and shrinkage of extensive initially unresectable primary tumors.
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PMID:The impact of chemotherapy and radiation therapy on secondary operations for neuroblastoma. 736 19

Pediatric malignant pheochromocytomas are very rare tumors, and no institution has more than one or two of these problem cases. The authors report on two children with such tumors, over a 9-year period, from two hospitals. In 1984, D.B. (14 years of age) presented with symptoms and signs of extradural metastasis from a right adrenal primary; he also had lung and bone metastases. After spinal decompression almost 4 years. He has remained well and is in remission 6 years later. In 1987, G.R. (13 years of age) presented with a larger right adrenal malignant pheochromocytoma invading surrounding structures; he also had liver metastasis. Preoperative chemotherapy did not shrink the tumor much; it was grossly resected, and there were many postoperative problems. In 1990, bone metastases developed, for which radiotherapy and chemotherapy were used. Three years later, the metastases have not disappeared; he remains on chemotherapy, and his liver function is borderline. From our small experience as well as a literature review, it appears that surgical excision remains the treatment of choice for the pediatric malignant phoechromocytoma. Unresectable tumors may be rendered resectable by intensive chemotherapy (similar to that used for neuroblastoma); adjuvant chemotherapy should be used for residual disease after surgery and for metastatic disease.
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PMID:Pediatric malignant pheochromocytoma. 780 44

From October 87 to April 92, 172 children were admitted in the N-I-87 protocol of the Spanish Society of Pediatric Oncology for the diagnosis and treatment of neuroblastoma. Forty-eight were considered Evans stage III, 33 of them being older than 1 year. All children were treated with induction chemotherapy (IC) and surgery. IC consisted of three courses of high-dose cisplatin-VM-26 alternating with three further courses of cyclophosphamide-doxorubicin (CAD). Infants less than 1 year received the same drugs at lower doses. After surgery, maintenance chemotherapy was administered to all children during 14 months. It consisted of four pairs of drugs rotated every 4 weeks. Radiotherapy was administered exclusively to patients older than 1 year with residual tumor after IC and surgery. Response was evaluated after IC and surgery. In children older than 1 year, response was obtained in 28/33 (88%). Fifteen of them (47%) achieved complete remission (CR), seven (22%) good partial response (GPR), six (19%) partial response (PR); and in three patients (9%) there was progressive disease (PD). Actuarial survival at 48 months was 0.60 +/- 0.10 and EFS was 0.61 +/- 0.12. Audiologic impairment was considered the worst toxicity. In children less than 1 year the response rate to IC and surgery was 93% (14/15); nine infants obtained complete response and four had GPR. Only one patient experienced PD in the first 6 months of therapy and died. The other 14 are alive and well at a mean follow-up time of 48 months. Chemotherapy toxicity was mild and reversible.
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PMID:Treatment of stage III neuroblastoma with emphasis on intensive induction chemotherapy: a report from the Neuroblastoma Group of the Spanish Society of Pediatric Oncology. 796 89

The use of differentiation-inducing agents has been proposed for the purging of bone marrow and for the treatment of minimal residual disease prior to autologous bone marrow transplantation in patients with metastatic neuroblastoma. The present studies examine the effects of the enediyne differentiation inducer neocarzinostatin (NCS) on tumor development from subcutaneous implants of murine (Neuro-2A) neuroblastoma cells. Prior in vitro treatment with NCS results in a concentration- and drug exposure time-dependent decrease in the incidence of tumors from subcutaneously implanted cells. In vivo treatment results in a dose-dependent decrease in the rate of tumor growth. These results imply that enediynes such as NCS may be useful in ex vivo purging regimens and in in vivo treatment of microscopic residual disease in patients with neuroblastoma.
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PMID:Effects of neocarzinostatin upon the development of tumors from murine neuroblastoma cells. 798 86

Patients with solid tumors are increasingly being treated by autologous bone marrow transplantation (BMT). Although response rates appear to be increased, disease recurrence is the commonest cause of treatment failure. Whether relapse is entirely due to residual disease in the patient or arises also from infiltrating malignant cells contained in the autologous marrow transplant has not been resolved. If the latter explanation is correct, then purging would be required as part of the transplantation procedure. We used retrovirally mediated transfer of the neomycin-resistance gene to mark BM harvested from eight patients with neuroblastoma in clinical remission. The marked marrow cells were subsequently reinfused as part of an autologous BMT. At relapse, we sought the marker gene in malignant cell populations. Three patients have relapsed, and in each the marker gene was detected by phenotypic and genetic analyses of resurgent malignant cells at medullary and extramedullary sites. Analysis of neuroblast DNA for discrete marker gene integration sites suggested that at least 200 malignant cells, each capable of tumor formation, were introduced with the autologous marrow transplant and contributed to relapse. Thus, autologous BMTs administered to patients with this solid tumor may contain a multiplicity of malignant cells that subsequently contribute to relapse. The marker-gene technique we describe should permit evaluation of the mechanisms of relapse and the efficacy of purging in patients receiving autologous marrow transplantation for other solid tumors that infiltrate the marrow.
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PMID:Direct demonstration that autologous bone marrow transplantation for solid tumors can return a multiplicity of tumorigenic cells. 802 66


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