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)

The clinical and pathologic findings of two patients with malignant ectomesenchymomas of soft tissue are reported. Malignant ectomesenchymomas are a composite of ganglion cells or neuroblasts and one or more malignant mesenchymal elements, usually rhabdomyosarcoma. Our first case was composed of ganglioneurosarcoma plus rhabdomyosarcoma, the second composed of neuroblastoma plus rhabdomyosarcoma. The name is derived from the suggestion that they arise from pluripotential ectomesenchyme. An English language literature search revealed 11 other cases that arose in soft tissue and had adequate clinicopathologic data. Of the 13 cases, 10 occurred in infants, three occurred in adults, and nine were males. Six patients were alive and free of disease at last follow-up (range, 0.6-12 years; mean, 3.4 years), four patients had died of tumor (within 0.5-3.3 years; mean, 1.3 years), one case had died of Adriamycin (doxorubicin) toxicity, and two cases had been lost to follow-up. Complete surgical resection is the mainstay of treatment and chemotherapy appears to be important.
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PMID:Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature. 295 Sep 92

A rationally devised induction regimen of vincristine, Adriamycin (Adria Laboratories, Columbus, OH), and sequentially-escalated cyclophosphamide (CPM), followed by S-phase-specific agents (5-fluorouracil [5-FU]/cytosine arabinoside [ara-C]/hydroxyurea), was used in 100 patients with neuroblastoma. Of 17 patients under 1 year of age at diagnosis, complete (CR)/good partial (GPR) responses with long-term disease-free survival were achieved in 11 (85%) of 13 new patients and in two of four previously treated patients; six of the GPRs also received myeloablative therapy with autologous bone marrow rescue to consolidate remission status. The 83 patients over 1 year of age at diagnosis included three groups: (1) 36 new patients whose N4SE included maximal-dose CPM (ie, up to 140 to 160 mg/kg/course); (2) an earlier group of 18 new patients whose N4SE included moderate-dose CPM (ie, up to 80 mg/kg/course); and (3) 29 previously treated patients who all received the maximal-dose N4SE regimen. For new patients, CR/GPR rates were 72% in the maximal-dose group v 39% in the earlier moderate-dose group (P = .029). A CR/GPR rate of 41% and a partial response rate of 17% were observed for the 29 previously treated patients, all but two of whom had large tumor burdens after therapy that included moderate doses of CPM. Despite consolidation with myeloablative therapy, many responders in the three groups ultimately relapsed. The N4SE was strongly myelosuppressive, but only two patients died from associated infection. Extramedullary toxicity was limited to hemorrhagic cystitis in four of 33 CPM previously treated patients; this problem did not occur in the 67 new patients. The data indicate that the maximal-dose N4SE is an effective induction regimen for neuroblastoma, can achieve marked regressions of disease resistant to less intensive therapy, and is sparing of major body organs. This high rate of remission induction must be coupled with improvements in consolidation therapy to assure long-term disease-free survival of poor-risk patients.
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PMID:Coordinated use of sequentially escalated cyclophosphamide and cell-cycle-specific chemotherapy (N4SE protocol) for advanced neuroblastoma: experience with 100 patients. 331 12

Since January 1983, 56 consecutive children over 1 year of age with stage IV neuroblastoma entered an aggressive protocol, including chemotherapy, radiation therapy, and bone marrow transplantation. The induction protocol included platinum and epipodophyllotoxin (VM-26), alternating with cyclophosphamide, Adriamycin (Adria Laboratories, Columbus, OH), and vincristine (PE/CADO). Surgery was performed after 2 to 4 months, and consolidation with intensive chemoradiotherapy and bone marrow transplantation (BMT) was performed within 12 months of diagnosis. The combination of vincristine, melphalan and total body irradiation (TBI) was used before BMT, and no further treatment was administered before progression. With the exception of two allografts, autologous BMT (ABMT) was given in all cases and was purged using an immunomagnetic procedure (Kemshead technique) in 32 of 35 cases, and a chemical procedure in three of 35. Of the 56 patients, 45 were evaluable. Of those, 23 were grafted in partial remission (PR), and 14 were grafted in either complete remission (CR) or very good partial remission (VGPR). The acute toxic death rate was 19%, the relapse rate was 32%, and the progressive disease rate was 19%. The progression-free survival in the CR/VGPR group (ie, 44% at 32 months post-diagnosis) and in the PR group (13% at 32 months) was not significantly different (P greater than .05). At 24 months, the overall survival of the 56 unselected patients was 39% compared with 12% for comparable patients previously treated by our group (P less than .005).
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PMID:High-dose chemoradiotherapy with bone marrow transplantation as consolidation treatment in neuroblastoma: an unselected group of stage IV patients over 1 year of age. 354 45

Anthracyclines such as Adriamycin (ADR) and daunomycin markedly inhibit cell growth in vivo and in vitro. These studies demonstrate that 30 microM hemin, which induces hemoglobin synthesis in human and murine erythroleukemia cells in culture, markedly decreases the cytotoxicity of ADR in a variety of hemopoietic cell lines (K562, HEL-1, MEL-745, HL-60, and U937) and in erythroid burst-forming cells from normal human marrow. Hemin failed to protect four of the five nonhemopoietic cell lines tested, including MCF-, breast adenocarcinoma cells, C-205 colon carcinoma cells, mouse 3T3 fibroblasts, and mouse kidney VERO cells. Hemin did protect human neuroblastoma IMP-32 cells from ADR cytotoxicity; however, this nonhemopoietic cell line undergoes dendrite formation in response to hemin induction. Cytofluorographic analysis of cellular ADR content and labeling studies with [3H]daunomycin demonstrated that hemin decreases the intracellular accumulation of these anthracyclines by more than 50% in K562 erythroleukemia cells. These studies indicate that small doses of hemin prevent intracellular accumulation of anthracyclines and thereby markedly reduce anthracycline toxicity to cells. Since this protective effect is observed preferentially with hemopoietic cells, it is possible that this finding could be exploited to protect the bone marrow from the cytotoxic action of anthracyclines during therapy for nonhemopoietic tumors.
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PMID:Prevention of anthracycline-induced cytotoxicity in hemopoietic cells by hemin. 370 75

The success rate of treatment for advanced neuroblastoma in childhood is still unsatisfactory. The Austrian pediatric oncology working group since 1979 uses a chemotherapy protocol of Dimethyl-triazeno-imidazol-carboxamid, Adriamycin, N-Lost, Vincristin or modified for age and stage a combination of Dimethyl-triazeno-imidazol-carboxamid, Adriamycin, N-Lost, Vincristin, Cyclophosphamide and Cis-platinum. Over a 30 month period 27 children with neuroblastoma were registered, six had a stage III, twelve a stage IV tumor. Currently 15 patients have no evidence of disease, six patients are alive with tumor, one patient with stage III and five patients with stage IV are dead.
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PMID:[Therapy of advanced neuroblastoma in children]. 618

Peripheral neuroepithelioma is a rare and controversial neoplasm that may occur at any age. Fifteen of the 38 previously reported cases have involved children from birth to 17 years of age. The authors observed the course of a 3-month-old girl who presented with an enlarging mass in the left arm and manifested hepatic metastases at the time of diagnosis. The urinary level of vanillylmandelic acid (VMA) was moderately elevated. The primary lesion was excised and metastatic foci showed response to a regimen of vincristine, cyclophosphamide, Adriamycin (doxorubicin), and cisplatin. However, tumor recurred in the brain and liver and the child died 14 months after diagnosis. At autopsy, there was no involvement of adrenal glands or sympathetic ganglia and the liver contained numerous involuted lesions as well as active metastases. It is suggested that this is a unique neoplasm, derived from neural crest but distinct from neuroblastoma, which can be characterized by peripheral origin, a histologic pattern of confluent pseudorosettes, and aggressive clinical behavior.
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PMID:Peripheral neuroepithelioma in childhood. 649 81

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

From 1975 to 1979, 173 children with neuroblastoma were treated according to the same protocol at the Institut Gustave-Roussy. They were classified according to the site of the primary tumor (abdominal: 122; thoracic: 29; others: 22) and according to TNM staging (stage I: 8; stage II: 24; stage III: 35; stage IV: 99; stage V: 2). Depending on stage and age, treatment consisted of surgery and radiotherapy associated with cyclic multiagent chemotherapy (vincristine, Adriamycin, cyclophosphamide). It resulted in a significant improvement of prognosis in stage III patients, especially those with abdominal tumors. In the latter group, prognosis depended mainly on the possibilities of resection of the tumors. Therefore, making these tumors operable remains the major goal of therapy in such patients. Radiotherapy is quite efficient in sterilizing the small post-surgical residual tumors. Prognosis in children over 1 year of age with metastases still remains very poor, even though the quality of the survival is improved.
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PMID:[Neuroblastomas treated at the Gustave-Roussy Institute from 1975 to 1979. 173 cases]. 686 66

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

A patient with olfactory neuroblastoma who had bone marrow metastasis at the time of diagnosis is presented. Previous therapy for this disease consisted of surgery and radiation. There is limited information relating to the efficacy of chemotherapy. Our patient was treated with combination chemotherapy (dacarbazine [DTIC-Dome], cyclophosphamide [Cytoxan], doxorubicin hydrochloride [Adriamycin], and vincristine sulfate [Oncovin]) and radiation to the primary site. Objective findings, more than two years after diagnosis, support a good partial response. Although a 50%, five-year survival time has been reported, the five-year cure rate is 18%. This report suggests that the role of combination chemotherapy should be further evaluated in certain patients with olfactory neuroblastoma.
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PMID:Olfactory neuroblastoma. Response to combination chemotherapy. 736 26


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