Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From September 1984 to March 1989, 57 children received intraoperative radiotherapy as part of a multidisciplinary tumor treatment. Their age ranged from 2 to 18 years. Tumor types: osteosarcoma, 21; Ewing's sarcoma, 19; soft tissue sarcomas, 6; neuroblastoma, 5; Wilm's tumor, 3; Hodgkin, 1; glioma, 1, and malignant pheochromocytoma, 1. In 44 patients the disease was localized while 13 had distant metastases. Intraoperative radiotherapy was used in 48 previously untreated patients as part of a radical treatment program and in 9 cases as an effort to rescue local failures (5 in previously irradiated areas). The intraoperative radiation field included the surgically exposed tumor or tumor bed, and the single doses ranged from 10 to 20 Gy, with 6-20 MeV electrons. With a median follow up time of 25 months (4 to 51 + months) 44 out of 57 patients are alive without local recurrence and 13 have died from tumor (6 with local progression). Intraoperative radiotherapy seems to be a feasible treatment which might promote local control in pediatric tumors.
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PMID:[Intraoperative radiotherapy in the multidisciplinary treatment of malignant tumors in children. Preliminary results]. 263 10

531 cases of malignant neoplasms and potentially malignant hyperplasia in children and adolescents diagnosed in the Department of Pathomorphology, Institute of Mother and Child between 1978/79 and 1983 are presented. Only patients treated in this hospital were included into the study. The most common diagnoses were neuroblastoma (68 cases), rhabdomyosarcoma (62 cases), nephroblastoma (56 cases), osteogenic sarcoma (47 cases), non-Hodgkin lymphoma (41 cases). Besides neoplasms most typical for childhood and adolescence the rare less typical cases were also seen.
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PMID:[Malignant neoplasms and potentially malignant hyperplasia in children and adolescents from material at the Department of Pathomorphology, Institute of Mother and Child during 1978/79-1983]. 263 66

In pediatric malignancies, particularly sarcomas, alkylating agents play an important role in the curative, combination chemotherapy approach. Since high doses of ifosfamide, the structural isomer of cyclophosphamide, were made tolerable with mesna uroprotection, high-dose ifosfamide (5 to 10 g/m2) has replaced conventional-dose cyclophosphamide (900 to 1,500 mg/m2) in some combination chemotherapy regimens. In Ewing's sarcoma and soft tissue sarcoma, the response rate and proportion of patients surviving disease free have been increased (at least for poor-prognosis patients) by 15% to 20% with an ifosfamide-containing regimen, as used in ongoing trials of the German Society of Pediatric Oncology. In germ cell tumors, the combination of ifosfamide and etoposide has proved to be an effective salvage regimen in patients resistant to vinblastine, bleomycin, and cisplatin. In stage IV disseminated neuroblastoma, however, the introduction of ifosfamide-containing regimens has not altered the poor prognosis. Results reflecting the value of ifosfamide in osteosarcoma, Wilms' tumor and Hodgkin's and non-Hodgkin's lymphomas are pending. It is not yet known whether high doses of akylating agents will increase the risk of late sequelae in cured patients.
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PMID:Ifosfamide in pediatric malignancies. 264 85

From September 1984 to July 1987, 33 children received intraoperative radiotherapy as part of a multidisciplinary tumor treatment. Their age ranged from 2 to 17 years. Tumors types: Ewing's sarcoma (n = 11), osteosarcoma (n = 8), soft tissue sarcomas (n = 5), Wilms' tumor (n = 3), neuroblastoma (n = 3), malignant pheochromocytoma (n = 1), Hodgkin's disease (n = 1), and optic nerve glioma (n = 1). In 25 patients the disease was localized while 8 had distant metastases. Intraoperative radiotherapy was used in 26 previously untreated patients as part of a radical treatment program and in 7 cases as an effort to rescue local failures (5 in previously irradiated areas). The intraoperative radiation field included the surgically exposed tumor or tumor bed, and the single doses ranged from 10 to 20 Gy, with 6-20 MeV electrons. Patients with osteosarcoma and recurrent tumor in a previously irradiated area did not receive postoperative external beam radiotherapy. With a median follow-up time of 10 months (1 to 31 + months) 24 out of 33 patients are alive without local recurrence and 9 have died from tumor (5 with local disease progression). Intraoperative radiotherapy seems to be a feasible treatment which might promote local control in pediatric tumors.
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PMID:Intraoperative radiotherapy in the multidisciplinary treatment of pediatric tumors. A preliminary report on initial results. 273 16

Postoperative course is reported in 52 children with malignant tumors (neuroblastoma, Wilms-tumor, non-Hodgkin-lymphoma, osteosarcoma etc.) who were operated on between 1979 and 1987. 26 children received chemotherapy prior to surgery, whereas 26 children were operated on without preceding chemotherapy (control group). Most children were under six years of age. 15 Children (57.7%) with preoperative chemotherapy developed early postoperative complications, such as sepsis, pneumonia, suture dehiscence, woundhealing disturbances and ileus, whereas this was the case in only 5 children (19.2%) without preoperative chemotherapy (P 0.0005). Four of the children with preoperative chemotherapy (15.4%) sustained late complications, such as local recurrence or mechanical bowel obstruction, whereas none of the control children did so. Lethality rate from underlying disease did not differ in both groups during follow-up (5 = 19.2% vs. 5 = 19.2%). This demonstrates that the surgeon must carefully be aware of an increased possibility of early and late complications in children who have to undergo surgery for malignant tumors following preoperative chemotherapy.
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PMID:[Postoperative course in children with malignant tumors following preoperative chemotherapy]. 273 47

The determination and comparison of genotypic combinations at genomic loci in normal and tumour tissues from patients with various types of cancer have defined the chromosomal locations of loci at which recessive mutations play a role in disease. The predisposing nature of some of these mutant alleles is exemplified in studies of retinoblastoma and osteogenic sarcoma. These two clinically associated diseases share a pathogenetically causal predisposition that maps to chromosome position 13q14. A similar mechanism at 11p15.5 is involved in the development of the embryonal variant of rhabdomyo-sarcoma, Wilms' tumour and hepatoblastoma. Finally, genomic alteration of chromosome 10 is apparent in glioblastomas and mixed tumours of glioblastoma/astrocytoma grade III but not in homogenous astrocytoma grades II or III, suggesting the definition of a locus involved in tumour progression and, perhaps, an approach to molecular genetic staging of tumours.
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PMID:Loss of genetic information in cancer. 274 36

A total of 20 children with recurrent or unresponsive tumours (10 Wilms' tumours, 3 rhabdomyosarcomas, 4 Ewing's sarcomas, 1 osteosarcoma, 1 hepatoblastoma, 1 hepatoma) were given ifosfamide as a 24-h infusion (5 g/m2), with mesna as a uroprotector. The number of courses ranged from 1 to 13 (median, 3), and the interval between them was 2-3 weeks. In all, 16 of these patients had previously received cyclophosphamide. Complete clinical responses (CRs) were seen in 3 cases (2 Wilms' tumours and 1 Ewing's sarcoma) and lasted 5, 7, and 9 months. Partial responses (PRs) were seen in 3 instances; mixed response or stable disease, in 4; and progressive disease, in 10. Treatment was well tolerated in most patients, with no cystitis or severe myelosuppression, but two children developed transient neurological symptoms and one became hypertensive. Nausea and vomiting were controlled by high-dose dexamethasone in most children.
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PMID:A phase II study of ifosfamide in paediatric solid tumours. 275 66

Among a cohort of 10,106 three-year survivors of childhood cancer, 90 second primary tumours (SPTs) were observed. Within 25 years of 3-year survival about 4% developed a SPT, about 6-fold expected, the relative risk not varying much with increasing follow-up. Following genetic retinoblastoma we observed 30-fold the expected number of SPTs, and over 400-fold the expected number of osteosarcomas. The risk of SPT in the absence of radiotherapy and chemotherapy (inherent risk) following genetic retinoblastoma was 13-fold expected and over 200-fold the expected number of osteosarcomas were observed. There was evidence that both radiotherapy and cyclophosphamide were associated with an increased risk of SPT. After all first primary tumours (FPTs) excluding retinoblastoma we observed almost 5-fold the expected number of SPTs. The inherent risk was 4-fold expected, the relative risks associated with radiotherapy but no chemotherapy, and both radiotherapy and chemotherapy were 6- and 9-fold expected respectively. There were about 20-fold the number of malignant bone tumours expected, most were osteosarcoma; also 7-fold the number of central nervous system tumours expected. There were 8 basal cell carcinomas and it seems likely that radiotherapy was involved in the development of some of these. Radiotherapy appears to have been involved in the development of many of the SPTs observed following all FPTs excluding retinoblastoma, particularly after CNS tumours, Wilms' tumour and Hodgkin's disease. Currently there is insufficient follow-up to examine the risk following chemotherapy. After acute leukaemia there was 20-fold the expected number of central nervous system tumours, though this is based on only 3 cases; whether therapy is directly involved in their development is uncertain. The risks we report are rarely greater than those reported in previous large-scale studies; in most instances they are substantially less. It is very unlikely that many SPTs were missed with our follow-up system so alternative explanations require further investigation; in particular it is possible the lower risks in our data compared to series treated in the United States may be explained, in part, by less combination therapy and lower doses of radiotherapy.
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PMID:Incidence of second primary tumours among childhood cancer survivors. 282 73

A new avian transforming retrovirus, NK24, was isolated from a chicken with a nephroblastoma. This transforming virus induced fibrosarcomas with osteogenic cell proliferation and nephroblastomas in vivo and transformed fibroblast cells in vitro. From extracts of NK24-transformed cells, anti-gag serum immunoprecipitated a 100-kilodalton nonglycosylated protein with no detectable protein kinase activity. An NK24 provirus present in infected quail cells was molecularly cloned and subjected to nucleotide sequence analysis. The genome of NK24 was 5.3 kilobases long and had a 1,126-base-pair sequence of cellular origin in place of a viral sequence of avian leukosis virus containing the 3' half of the gag gene and the 5' half of the pol gene. Although the entire env gene was retained, it appeared to be inactive, possibly owing to the loss of function of its splice acceptor site as a result of a second deletion of 1,598 bases in the 3' half of the pol gene that extended to the acceptor site. Nucleotide sequence analysis revealed that the NK24 virus contained the fos gene, previously identified as the oncogene of FBJ and FBR murine osteosarcoma viruses. Unlike the v-fos gene products of FBJ and FBR, which suffer a structural alteration at their carboxyl termini, the NK24 v-fos gene product seemed to have the same carboxyl-terminal structure as the chicken c-fos gene product. A comparison of the structures of the products of the NK24 v-fos and mouse c-fos genes suggested that the fos gene product consists of highly conserved regions and relatively divergent regions.
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PMID:An avian transforming retrovirus isolated from a nephroblastoma that carries the fos gene as the oncogene. 282 11

A retrospective analysis, from 1965 to 1987, of 22 patients less than 18 years of age undergoing thoracotomy for pulmonary metastases from previously diagnosed malignancy, was performed. There were 15 males and seven females whose ages ranged from 8 months to 17, years. Ten patients had primary osteogenic sarcoma, five had Wilms' tumor, and seven had miscellaneous other tumors. A total of 41 thoracotomies were performed with no mortality. The overall survival rate was 54.5%, with an average survival of 6.2 years after initial diagnosis. The osteosarcoma group had a 50% survival rate after an average of 62 months from initial diagnosis, while the Wilms' tumor group had an 80% survival rate with a 100-month average. The remaining seven patients had a 29% average survival rate 62 months after diagnosis. Of the 12 patients undergoing wedge resections, two died upon follow-up 20 and 21 months after initial diagnosis. As opposed to the survivors in this group, both required more than four wedge resections upon initial thoracotomy. Two patients requiring extended resections, one for Ewing's sarcoma and one for hepatoblastoma, died 35 and 3 months after diagnosis, respectively. Of the eight patients undergoing lobectomy and/or segmentectomy, 75% died an average of 31.3 months after diagnosis. Ten patients had two or more thoracotomies for an average of 2.9, with a 40% survival rate. Of the 27% who presented with initial bilateral lung metastases, 33% survived. Forty-five percent of patients had a tumor-free interval of less than 12 months prior to thoracotomy, resulting in a 60% mortality rate.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pulmonary metastases in children: results of surgical treatment. 284 44


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