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

An alarmingly high rate of postirradiation sarcomas following treatment for retinoblastoma has been described in the literature. We present four new cases and report 57 others from the English literature. Osteogenic sarcoma was the predominant histologic type (58%), followed by fibrosarcoma (21%) and various other sarcomas (21%). The average latency period between irradiation and development of the second primary (sarcoma) was 12.4 years. Irrespective of irradiation, a genetic linkage between retinoblastoma and osteogenic sarcoma on the 13q14 chromosome is recognized. Through a pleiotropic effect of this same chromosome, a predisposition for other sarcomas may exist as well. Finally, a strong role for radiation induction is proposed for all of these postirradiation sarcomas. This is based on the increased number of sarcomas arising in the field of prior irradiation (sites uncharacteristic of spontaneously occurring primary sarcomas) and the prolonged latency periods.
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PMID:Postirradiation sarcoma in retinoblastoma. Induction or predisposition? 328 48

Osteosarcoma is the most common bone tumor of children and adolescents. The peak incidence of the disease is in the 15 to 19 year age group. The disease is more commonly seen in males than females. While several factors, including exposure to radiation, genetic disorders such as retinoblastoma, and high rate of bone growth, have been associated with osteosarcoma, in most cases no definite etiology can be established. Osteosarcoma usually originates in the metaphyseal region of long bones and extends through the cortex, causing varying degrees of bone destruction and expansion of periosteum. The radiographic appearance caused by this process is often referred to as "sun burst" sign. Positive diagnosis of osteosarcoma is made by histopathology. The histopathological classification of osteosarcoma can also predict the degree of aggressive behavior of this tumor and thus has prognostic significance. Surgery, including amputation or limb-salvage procedure, is the mainstay of treatment of osteosarcoma. It is now unequivocally established that adjuvant chemotherapy will prolong the survival of patients with this disease. Chemotherapy agents often used include platinum derivates, methotrexate, vincristine, cyclophosphamide, adriamycin, actinomycin D, bleomycin and DTIC. Depending on surgical decision, these agents can be used prior to or after the operation. Immediate fitting with prosthesis and provision of appropriate medical and psychological support in the care of these patients is essential.
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PMID:Osteosarcoma. 329 Aug 15

A strong case for a genetic diathesis in human cancer can be made through the analysis of familial aggregations of mixed tumor types. This is particularly true for childhood cancers which have a very low incidence when compared to that of adult cancer and for which common environmental factors appear to play little etiological role. Here are described studies designed to apply molecular genetic analysis toward defining the lesions which predispose to human cancer. We found that the clinically associated tumors retinoblastoma and osteosarcoma share a pathogenetic mechanism entailing aberrant chromosomal segregation events during mitosis which lead to tumor cells homozygous for recessive mutant alleles at the RB1 locus on human chromosome 13 band q14. These results suggest that a rational explanation for the sequential occurrence in these children of two different tumor types is the initial inheritance of a predisposing recessive mutation with broad but specific tissue activity.
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PMID:Chromosomal mechanisms in the initiation of human familial mixed cancers. 333 9

An osteosarcoma cell line, OHS, was established from a patient with multiple skeletal manifestations of osteosarcoma, developing after bilateral retinoblastoma. The tumor cells expressed sarcoma-associated antigens and showed rapid growth in monolayers and as multicellular spheroids. They formed distinct colonies in soft agar, and subcutaneous tumors in nude mice. Morphological studies indicated that OHS cells had retained important characteristics of the cells of origin. No deletion of the retinoblastoma genes on chromosome 13q14 could be demonstrated with the banding techniques used. However, cytogenetic studies revealed double minute chromosomes, as evidence of gene amplification, as well as translocations involving chromosomes 1,6,11 and 13. The OHS line can be used to study the genetic basis of tumor initiation and growth, and to elucidate factors predisposing for second primary cancers in retinoblastoma patients.
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PMID:Characteristics of a cell line established from a patient with multiple osteosarcoma, appearing 13 years after treatment for bilateral retinoblastoma. 345 16

A case of osteosarcoma of the distal femur 13 years after the occurrence of a unilateral nonhereditary retinoblastoma is reported. The karyotype of circulating lymphocytis was found to be normal. The existence of a linkage between the two neoplasms due to a subtle chromosomal abnormality is discussed.
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PMID:Osteosarcoma as a primary second tumor in a patient with nonhereditary retinoblastoma. 346 13

One hundred and sixty one children who have developed more than one primary neoplasm have been identified. Children with tumours of the central nervous system, retinoblastoma and leukaemia were those most frequently observed to develop a second malignancy whilst osteosarcoma was the most common second tumour. The patterns of second neoplasms appear to be changing and a recent increase in the number of children with leukaemia and lymphoma who develop second primary tumours has been observed. In this series, the two most frequent associations of tumours were retinoblastoma followed by osteosarcoma and the combination of acute leukaemia with a tumour of the central nervous system. Genetic factors which may have contributed to the development of the second primary tumour were identified in 53 patients (33%), 33 of whom had the genetic form of retinoblastoma. In an analysis of the treatment of 151 patients, for whom the interval between the two neoplasms was greater than 12 months, the second malignancy was considered to be 'radiation associated' in 93 (61%). Fifty children (33%) had been treated with either single or multiple agent chemotherapy which included an alkylating agent in 38. Forty five children had received a combination of chemotherapy and radiotherapy and of these, 10 developed leukaemia as their second tumour. Of the 19 secondary leukaemias, 16 have occurred in patients treated since 1970.
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PMID:Patterns of multiple primary tumours in patients treated for cancer during childhood. 347 72

The genetic predisposing factor for childhood retinoblastoma resides on the q14 band of human chromosome 13. However, a postulated second genetic event must take place for the disease to occur, which further research indicates involves inactivation of the remaining functional allele within 13q14. Our isolation of the gene within 13q14, called Rb, required creating a lambda-phage library that contained inserted fragments from human chromosome 13. One of the inserts, H3-8, detected a corresponding 1.8-kb HindIII fragment that was deleted in 2 of 37 retinoblastoma tumor DNAs. This suggested that the probed segment was linked to the Rb gene. A nearby probe, p7H30.7R, detected not only the human sequence but also a mouse homologue in a somatic cell hybrid carrying human chromosome 13. We used the p7H30.7R probe for RNA analysis to detect any transcripts in a retinal cell line. The analysis showed a 4.7-kb transcript in the tumor cell line but not in several retinoblastomas. We also failed to detect a transcript in four retinoblastoma and two osteosarcoma samples using a corresponding cDNA fragment termed p4.7R. We used this probe to analyze the DNA from a large group of retinoblastomas and osteosarcomas and found gross changes in genomic structure in approximately 30% of the tumor DNAs. The boundaries of homozygously deleted fragments were mapped. In the analysis of an osteosarcoma and a retinoblastoma we discovered that the endpoints of the deletions were within the confines of the genetic unit defined by the probe. This indicated that the target of inactivation was the segment under study and not a neighboring DNA sequence.
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PMID:Genetic sequences that predispose to retinoblastoma and osteosarcoma. 348 May 47

Seven cases of malignant melanoma in the close relatives of children with osteosarcoma and chondrosarcoma are described. The association between certain childhood malignancies (adrenal cortical carcinoma, osteosarcoma, chondrosarcoma, retinoblastoma) and malignant melanoma is discussed and it is proposed that in certain families malignant melanoma may be another manifestation of the same gene defect which results in susceptibility to tumours characteristic of the SBLA cancer family syndrome.
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PMID:Malignant melanoma in families of children with osteosarcoma, chondrosarcoma, and adrenal cortical carcinoma. 348 Sep 57

Studies of the presenting height of children with malignancies have produced conflicting results, from an excess of taller patients to an excess of shorter patients. The problems of measurement bias, inadequate comparison populations, small numbers of patients, subgroup analyses, and overreliance on simple significance tests are all possible reasons for the variation in results. To clarify this issue, we studied heights at diagnosis of 3657 children and adolescents aged under 18 years. Their malignancies included acute lymphoblastic leukaemia, non-Hodgkin's lymphoma, Hodgkin's disease, acute non-lymphoblastic leukaemia, osteosarcoma, retinoblastoma, neuroblastoma, Wilms' tumour, rhabdomyosarcoma, and Ewing's sarcoma. Compared with published standards for the heights of children in control populations, no significant deviation from population norms was found for patients in any of the 10 disease categories after proper adjustment for multiple significance testing.
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PMID:Height at diagnosis of malignancies. 360 84

In a series of 882 retinoblastoma patients, 384 known to have the genetic form of the disease and 498 others, 30 patients developed second primary neoplasms. The spectrum of these second neoplasms is discussed in relation to the forms of treatment used for the retinoblastoma. Cumulative incidence rates of second tumours in the whole series are 2.0% at 12 years after diagnosis and 4.2% after 18 years. For patients with the genetic form of retinoblastoma the cumulative incidence rate after 18 years is 8.4% for all second neoplasms and 6.0% for osteosarcomas alone. The inherent risk among survivors from genetic retinoblastoma of developing an osteosarcoma, excluding all possible effects of treatment, is estimated to be 2.2% after 18 years. Within the field of radiation treatment the cumulative incidence rate for all second neoplasms after 18 years is 6.6% and for osteosarcomas alone 3.7%. There is some evidence that patients with genetic retinoblastoma are particularly sensitive to the carcinogenic effects of radiation. The results also suggest that the use of cyclophosphamide may increase the risk of second primary neoplasms in patients with genetic retinoblastoma. The incidence rates of second primary neoplasms in retinoblastoma survivors reported here are lower than those quoted for previously published series. Evidence from this and other papers strongly suggests an association between retinoblastoma and malignant melanoma.
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PMID:Second primary neoplasms in patients with retinoblastoma. 371 23


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