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)

Second malignancy after childhood neoplasms is a well-known complication. However, frequency differs considerably according to the types of primary neoplasm and the specifics of therapy. Ten patients with a second malignancy after being cured of the primary tumor are described. There were 2 patients with acute lymphoblastic leukemia, one with non-Hodgkin's lymphoma, and one with breast cancer after Hodgkin's disease. Two patients with heritable retinoblastoma developed osteosarcomas in the irradiation field after a latent period of 7 and 14 years respectively. There was another osteosarcoma in a Wilms' tumor survivor. One patient with acute lymphoblastic leukemia developed a secondary AML 10 years after achieving initial remission, and a meningioma was diagnosed in another patient with cured acute lymphoblastic leukemia. One patient died of peritoneal sarcomatosis of unknown origin 20 years after the diagnosis of acute myeloid leukemia. All patients received radiotherapy for the primary neoplasms. Secondary neoplasms in other patients were probably missed because they occurred in adulthood when the patients were transferred to other medical centres. It is impossible to trace these patients because central registration of patients with neoplasms is lacking. It is therefore important to establish a central cancer registry for the whole of Switzerland. Second malignancy after childhood cancer is not a rare event and requires long-term follow-up of patients with neoplasms.
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PMID:[Insufficient understanding of second tumors after childhood neoplasms in Switzerland]. 958 99

The purpose of this study was to provide an overview of the spectrum of pediatric chest masses, to present the results of cross-sectional imaging with CT and/or MRI, and to define diagnostic criteria to limit differential diagnosis. Seventy-eight children with thoracic mass lesions were retrospectively evaluated using CT (72 patients) and/or MR imaging (12 patients). All masses were evaluated for tissue characteristics (attenuation values or signal intensity, enhancement, and calcification) and were differentiated according to age, gender, location, and etiology. Twenty-eight of 38 (74 %) mediastinal masses were malignant (neuroblastoma, malignant lymphoma). Thirty of 38 (79 %) pulmonary masses were metastatic in origin, all with an already known primary tumor (osteosarcoma, Wilms tumor). With one exception, all remaining pulmonary lesions were benign. Seventeen of 21 (81 %) chest wall lesions were malignant (Ewing sarcoma, primitive neuroectodermal tumor). The majority of mediastinal and chest wall tumors in children is malignant. Lung lesions are usually benign, unless a known extrapulmonary tumor suggests pulmonary metastases. Cross-sectional imaging with CT and/or MRI allows narrowing of the differential diagnosis of pediatric chest masses substantially by defining the origin and tissue characteristics. Magnetic resonance imaging is preferred for posterior mediastinal lesions, whereas CT should be used for pulmonary lesions. For the residual locations both modalities are complementary.
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PMID:Cross-sectional imaging with CT and/or MRI of pediatric chest tumors. 968 16

The Wilms' tumor suppressor gene, wt1, encodes a zinc-finger transcription factor, WT1, that represses transcription of a number of growth-promoting genes and inhibits cell growth. The transcripts of wt1 undergo two alternative splicing events, giving rise to four isoforms of mRNA in constant ratios. The first alternative splice introduces an extra exon 5, which encodes 17 amino acid residues inserted between the transcription regulatory domain and the DNA binding domain of WT1. Previously, we demonstrated that the 17-amino acid domain functioned as a transcription repressor when it was fused with the DNA binding domain of WT1. We have now identified a point mutation within exon 5 of wt1 in a sporadic unilateral Wilms' tumor patient. The mutation changes the last of the 17 amino acids from asparagine to serine. The protein isoform of WT1 carrying this mutation exhibited a 2-3-fold lower transcription-repressing activity than wild-type WT1 in transient cotransfection assays. The mutation also decreased growth-inhibiting activity of WT1 in two osteosarcoma cell lines, U2OS and Saos-2. By diminishing transcription-repressing and growth-inhibiting activities of WT1, this naturally occurring mutation within exon 5 of wt1 may disturb the normal function of the protein and lead to the uncontrolled cell growth characteristic of Wilms' tumor.
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PMID:A point mutation within exon 5 of the WT1 gene of a sporadic unilateral Wilms' tumor alters gene function. 975 32

Childhood cancer often results in pulmonary metastases, and proper identification and prompt treatment of these lesions increases the survival of these patients. Between 21 May 1995 and 26 June 1996, nine children (five with osteosarcoma, two with Wilms' tumor, and one each with hepatoblastoma and an adrenocortical tumor) underwent median sternotomy at our institution for resection of bilateral pulmonary metastases. These five boys and four girls ranged in age from 2 to 16 years (median, 11 years). Identification and resection of metastases in all lobes of the lungs was greatly facilitated by the median sternotomy approach because both thoracic cavities were fully accessible. In each of five patients, as many as 23 metastases were removed using wedge resection or a stapling device. Multiple biopsies only were obtained from the four remaining patients, whose lesions were too numerous and diffuse for effective resection. All nine patients recovered uneventfully; the median hospitalization was 3 days (range, 2-5 days). We find that median sternotomy is a safe, effective, and relatively simple procedure for resecting bilateral pulmonary metastases in children with cancer.
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PMID:Median sternotomy for the resection of bilateral pulmonary metastases in children. 979 75

Aging is the gradual development of individuals of a particular species in time. Interspecies comparison shows the variability of the aging processes in ontogeny which is species-specifically fixed. e.g. a mouse will never be able to reach the life expectancy of a blue whale or man. Certain changes of the life span have been observed in aging human populations, on an individual basis, or in zoo animals under the care of veterinarians. Cells of different tissues exhibit a varying senescence additionally influenced by concomitant diseases. The age spectrum of neoplasms cannot be simplified. It is misleading to state that neoplasms are diseases of the elderly because this is the case only in certain, very common neoplastic diseases, such as colon cancers, but not regarding pediatric neoplasms as retinoblastoma, nephroblastoma, or Ewing sarcoma, and osteosarcoma in the young. This paper evaluates the diversified age changes in the development of selected neoplastic diseases.
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PMID:Aging and neoplastic progression. 989 Dec 33

Seasonal trends in month of diagnosis have been reported for childhood acute lymphoblastic leukaemia (ALL) and non-Hodgkin's lymphoma (NHL). This seasonal variation has been suggested to represent an underlying viral aetiology for these malignancies. Some studies have shown the highest frequency of diagnoses in the summer months, although this has been inconsistent. Data from the Children's Cancer Group and the Pediatric Oncology Group were analysed for seasonal incidence patterns. A total of 20,949 incident cancer cases diagnosed in the USA from 1 January 1989 through 31 December 1991 were available for analyses. Diagnosis-specific malignancies available for evaluation included ALL, acute myeloid leukaemia (AML), Hodgkin's disease, NHL, rhabdomyosarcoma, neuroblastoma, retinoblastoma, osteosarcoma, Wilms' tumour, retinoblastoma, Ewings' sarcoma, central nervous system (CNS) tumours and hepatoblastoma. Overall, there was no statistically significant seasonal variation in the month of diagnosis for all childhood cancers combined. For diagnosis-specific malignancies, there was a statistically significant seasonal variation for ALL (P = 0.01; peak in summer), rhabdomyosarcoma (P = 0.03; spring/summer) and hepatoblastoma (P = 0.01; summer); there was no seasonal variation in the diagnosis of NHL. When cases were restricted to latitudes greater than 40 degrees ('north'), seasonal patterns were apparent only for ALL and hepatoblastoma. Notably, 33% of hepatoblastoma cases were diagnosed in the summer months. In contrast, for latitudes less than 40 degrees ('south'), only CNS tumours demonstrated a seasonal pattern (P = 0.002; winter). Although these data provide modest support for a summer peak in the diagnosis of childhood ALL, any underlying biological mechanisms that account for these seasonal patterns are likely complex and in need of more definitive studies.
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PMID:Seasonal variations in the diagnosis of childhood cancer in the United States. 1094 15

Survival following cancer was analysed in relation to ethnic group among children diagnosed in Britain during 1981-1996 and treated at paediatric oncology centres by members of the UK Children's Cancer Study Group. Survival was analysed for 11 diagnostic groups: acute lymphoblastic leukaemia (ALL), acute non-lymphocytic leukaemia, Hodgkin's disease, non-Hodgkin's lymphoma, astrocytoma, primitive neuroectodermal tumour, neuroblastoma, Wilms' tumour, osteosarcoma, Ewing's sarcoma and rhabdomyosarcoma. There were no significant differences in survival between White and non-White children over the study period as a whole. Among children with ALL, however, the relative risk of death allowing for period of diagnosis, age and white blood count was 1.25 for those of South Asian ethnic origin compared with Whites (P = 0.057).
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PMID:Ethnic group and survival from childhood cancer: report from the UK Children's Cancer Study Group. 1075 11

The Hungarian Pediatric Oncology Working Group intended to change the practice of prescribing diagnostic tests as well as to examine the possibility of introducing indicators about the time factors of medical care. A nationwide accepted protocol was established for these tests. The examined time factors were the length of elapsed time from admittance to treatment and the length of hospital stay for different reasons (diagnosis, treatment, complications). Included into this study are the new cases of 5 common groups of malignancies (acute lymphoblastic leukemia and non-Hodgkin lymphoma, osteosarcoma, soft tissue sarcoma, Wilms tumor, neuroblastoma) for a study period of 1 year. The follow-up data of 152 patients were examined; the length of survey was 1-12 months, depending on when the patients entered the study. As a continuous clinical audit, a system of survey was set up for each follow-up test, using a questionnaire about the conformity of physicians to the protocol, evaluating the principal reasons of deviation from the protocol. Using the data provided by this questionnaire, a renewal of the protocols for each disease was made three times during the whole study period. The principal reasons of nonconformity to the protocol were (1) complications, (2) the nonuse of the protocol, (3) nonacceptance of the protocol, and (4) technical problems. The authors intended to use their time indicators for benchmarking, to make a comparison possible between centers concerning the length of treatment, occurrence of complications, and delays in chemotherapy. However, the examination of the time indicators in the most frequent disease (acute lymphoblastic leukemia, n = 73) showed inverse correlation between the number of admissions per year per center and the length of time elapsed up to the beginning of treatment. This points to a need for better cooperation in small centers at the initial phase of the diagnosis. The main result of this study is the successful elaboration and implementation of practice guidelines by information linked to performance (the feedback) in daily practice: Compliance during the first 3 months of the study was 28%, and compliance during the last 3 months was 61%.
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PMID:Practice guidelines in pediatric hematooncology: implementation and survey. A possible way for medical quality assurance. 1112

According to data available at the Institute (1985-1999, approx. 1,200 patients), the lung is the most frequent site of cancer dissemination (up to 75%). Such most frequent pediatric children tumors as Wilms' tumor, neuroblastoma, rhabdosarcoma, osteogenic sarcoma and that of Ewing disseminate to lung in 85%, particularly, at advanced stages.
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PMID:[Metastatic lung tumors in children (clinical course, diagnosis, prognosis)]. 1114 22

The spectrum and frequency of cancers associated with germline TP53 mutations are uncertain. To address this issue a cohort of individuals from 28 families with Li-Fraumeni syndrome, segregating germline TP53 mutations was established. Predicted cancers were estimated by applying age, morphology, site and sex-specific UK cancer statistics to person-years at risk. Observed and predicted cancers were compared and two-sided P-values calculated. Cancer types occurring to excess and showing P-values <0.02, were designated strongly associated with germline TP53 mutations. These were removed from the data and a second round of analyses performed. Cancer types with P-values <0.02 and 0.02-0.05 in the second round analyses were considered moderately and weakly associated respectively. Strongly associated cancers were: breast carcinoma, soft tissue sarcomas, osteosarcoma, brain tumours, adrenocortical carcinoma, Wilms' tumour and phyllodes tumour. Carcinoma of pancreas was moderately associated. Leukaemia and neuroblastoma were weakly associated. Other common carcinomas including lung, colon, bladder, prostate, cervix and ovary did not occur to excess. Although breast carcinoma and sarcomas were numerically most frequent, the greatest increases relative to general population rates were in adrenocortical carcinoma and phyllodes tumour. We conclude that germline TP53 mutations do not simply increase general cancer risk. There are tissue-specific effects.
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PMID:Relative frequency and morphology of cancers in carriers of germline TP53 mutations. 1149 85


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