Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The purpose of this paper was to define the histologic distribution, clinical features, and treatment response of childhood non-Hodgkin lymphoma (NHL) in northeastern Brazil. We reviewed medical records and histopathologic studies of 98 children treated for NHL from 1980 to 1987 at a major pediatric cancer center in Recife, Brazil. Treatment outcome was evaluated in relation to tumor burden (stage and serum LDH) and type of therapy (LSA2L2 vs other multiagent chemotherapy). There was a striking predominance of the small noncleaved cell (Burkitt) subtype, which occurred in 92 of the 98 children and adolescents diagnosed with NHL. Subsequent analyses focused on these patients. The majority (n = 84) had advanced (stage III/IV) disease at diagnosis. The abdomen was the most common site of disease (84 cases); jaw involvement was rare (three cases). Five-year event-free survival (excluding treatment refusals) was significantly better for patients with limited vs advanced stage disease (75 +/- 14% vs 42 +/- 6%; P < 0.04). Elevated serum LDH (>500 U/l) was associated with a poorer outcome (P = 0.008). The type of chemotherapy did not affect EFS (P = 0.95). Only 39% of patients are long-term survivors, reflecting the high rate of septic deaths (25% of patients) and parental refusal/abandonment of therapy (10%). Epstein-Barr virus (EBV) was detected in tumor cells from eight of the 11 cases studied. In clinical presentation, these cases resemble sporadic Burkitt lymphoma, yet in their apparent responsiveness to LSA2L2 therapy and association with EBV, they do not. Childhood NHL in northeastern Brazil is predominantly of the Burkitt subtype, and is associated with clinical features that appear to distinguish it from the endemic and sporadic forms of this tumor. These cases may represent a third or intermediate subtype of Burkitt lymphoma.
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PMID:Predominance and characteristics of Burkitt lymphoma among children with non-Hodgkin lymphoma in northeastern Brazil. 918 Mar 1

Second malignant neoplasms are an infrequent but well-documented sequelae of radiation therapy for childhood cancer. We report a 34-year-old man with chondrosarcoma of the spine and thyroid carcinoma diagnosed 24 years after radiation therapy for Hodgkin's lymphoma. Both tumors arose in the previously irradiated field and were not detected until the patient presented with paraplegia. The propensity of these neoplasms to arise in the previously irradiated field warrants physicians to be alert to any manifestations arising in this anatomic area.
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PMID:Chondrosarcoma of the spine and thyroid carcinoma following radiation therapy for Hodgkin's lymphoma. 919 92

Cancer is an infrequent disease in childhood. However, it is responsible for 13.06% of child deaths between 1 and 14 years in Cuba. The aim of our work was to describe the incidence of childhood cancer in the period 1986 to 1990 using data reported to the National Cancer Registry (NCR) of Cuba. All cancer cases between 0 and 14 years of age reported to the NCR in the period 1986 to 1990, were included. The cases were classified histologically, by age and gender. Average age-specific and age-standardized rates were calculated; 1428 children were registered, an average of 286 each year, with a mean annual rate of 117.8 per million. The world-population standardized rate was 120.7 and the male-to-female ratio was 0.98. Leukaemias, lymphomas and malignant tumours of the central nervous system were the most common childhood neoplasm groups. The majority of leukaemias were acute lymphoblastic leukaemias (ALL), and the incidence peak extended until 6 years of age. The first peak of incidence of Hodgkin's disease is found in older age-groups. Burkitt's lymphoma showed a male-to-female ratio of 0.44. Most of the hepatic tumours were carcinomas, and only one fourth were hepatoblastomas. In bone tumours, similar rates were observed for osteosarcomas and Ewing's sarcoma.
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PMID:Incidence of childhood cancer in Cuba (1986-1990). 925 90

Prophylactic treatment of the central nervous system (CNS) in childhood leukemia and non-Hodgkin lymphoma (NHL) has negative effects on intelligence. We investigated the clinical significance of this finding by comparing the effect of different types of CNS prophylaxis on the survivor's learning capabilities. To isolate the effect of different types of CNS prophylaxis from other treatment and disease variables on learning problems, children treated for leukemia or NHL who received CNS prophylaxis with cranial irradiation (n = 30) or without cranial irradiation (n = 36) were compared with children treated for solid tumors who received systemic chemotherapy without any CNS treatment (n = 30) and with matched healthy controls (n = 265). The identification of learning problems was based on the school system's assessment. Parents and teachers reported on the child's educational status in a standardized way. Learning problems were found in 80% of children who received CNS prophylaxis with cranial irradiation. This was significantly higher than the 14% found in children treated with CNS prophylaxis without cranial irradiation (P < 0.000). The prevalence of learning problems in this latter group did not differ significantly from that in childhood cancer survivors without any form of CNS prophylaxis (20%) and in healthy matched controls (17%). We conclude that the high prevalence of learning problems in survivors of childhood leukemia and NHL is directly related to CNS prophylaxis with cranial irradiation and not to CNS prophylaxis per se or to other treatment and disease variables.
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PMID:Cranial irradiation is the major cause of learning problems in children treated for leukemia and lymphoma: a comparative study. 926 69

Patterns and trends in childhood cancer incidence for Navarre and Zaragoza registries were studied over the 15-year period, 1973-1987. Overall cancer rates and rates for 10 specific types of cancer were analysed using a log-linear Poisson model or, alternatively, a gamma-Poisson model whenever overdispersion was present, with age, sex, registry and period being used as predictor variables. Childhood cancer was 30% more frequent in boys than in girls, and, except for lymphomas and bone tumours, incidence decreased remarkably with age. Adjusted rates were high in comparison with other European countries, particularly in the case of non-Hodgkin's lymphomas. Cancer rates proved somewhat higher in Navarre, but this difference attained statistical significance solely in the case of central nervous system tumours (rate ratio = 1.75; 95% confidence interval 1.21-2.54). A significant rise in overall incidence was observed (11% 5-yearly increase) due mainly to the upward trend in central nervous system tumours. While the rise in these tumours coincides with the period which witnessed the spread of computerised tomography in Spain, the trend nevertheless held steady over the last 5-year period, when access to this diagnostic technique had already become generalised nationwide.
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PMID:Childhood cancer incidence in Zaragoza and Navarre (Spain): 1973-1987. 927 44

The Chernobyl accident in 1986 had a major ecological impact, with the bulk of the radioactive contamination affecting Belarus, the Ukraine and Russian Federation. Belarus has a nationwide general cancer registry that dates back to 1965, which allows a comparison of cancer incidence rates from before and after the accident. Preliminary analysis indicates that there has been an increased incidence of all cancers, with thyroid cancer accounting for most of that change. When cancer incidence data from Belarus are compared to data from the U.S., there is a higher incidence of thyroid cancer and a slightly higher incidence of Hodgkin's disease and non-Hodgkin's lymphoma in Belarus, but a lower reported incidence of leukemia and brain tumors. The Belarusian State Cancer Registry is being used as a foundation for the development of a more comprehensive childhood cancer registry.
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PMID:Epidemiology of childhood cancer in Belarus: review of data 1978-1994, and discussion of the new Belarusian Childhood Cancer Registry. 936 8

The use of RT in pediatric cancer has been virtually eliminated in certain diseases (NHL); greatly reduced in some (Wilms' tumor, ALL, neuroblastoma); and refined and modified in others (rhabdomyosarcoma, Ewing's sarcoma). At present, however, it seems clear that RT will continue to be an important modality (particularly in brain tumors) and a much greater understanding of its effects has been achieved and utilized. The knowledge of the occurrence of late effects and SMN in a child cured of cancer is continuing to modify initial treatment strategies: A classic example of such an effort is the common use of lower RT doses and nonalkylator-based chemotherapy in Hodgkin's disease. Further, the use of DNA testing in children may be able to identify the presence of germline RB and p53 mutations, which may identify a child at high risk for SMN, so that appropriate therapeutic modifications may be made. In addition, knowledge of these late consequences in children mandates that they be carefully monitored and closely followed, so that prompt and effective treatment can be administered to give them a better chance for a long and healthy life.
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PMID:Pediatric radiotherapy. An overview. 937 90

A retrospective analysis of 515 pediatric cancer cases diagnosed over 18 years, 1973-1990, showed an annual incidence of pediatric solid tumors in northern Israel of 77.1 per million, somewhat lower than previously reported. Lymphomas predominated over central nervous system (CNS) neoplasms, suggesting an Afro-Asian rather than a Western pattern. Jewish and non-Jewish children were at approximately equal risk (1:07:1.0) for the nonleukemic cancer. However, there was a notably higher frequency in males than females (1:42:1.0) and in Ashkenasi Jews as compared to either Sephardi Jews (1.25:1.00) or non-Jews (1.23:1.0). Ethnic, age, and sex predispositions for particular types of malignancy were also noted. Non-Jews tended to have lymphomas or retinoblastomas and Sephardi Jews were predisposed to soft tissue sarcomas. Ashkenasi Jews tended to manifest CNS tumors, retinoblastoma, and osteosarcoma. Children under 5 years showed Burkitt's lymphoma and neuroblastoma, whereas the older group tended to have Hodgkin's lymphoma. Boys were more vulnerable to non-Hodgkin's lymphoma, medulloblastoma, neuroblastoma, and rhabdomyosarcoma, and girls were subject to higher incidences of bone, gonadal, germ cell, and epithelial tumors, as well as to astrocytoma. The implications for genetic or environmental contributions to several cancers are considered in conjunction with ethnic or gender predisposition to those cancers.
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PMID:Patterns of childhood solid tumor incidence in northern Israel, 1973-1990. 938 5

Mutant frequencies (Mfs) at the two genetic loci, the hypoxanthine phosphoribosyl transferase (hprt) gene and the T-cell receptor (TCR) gene were evaluated in pediatric cancer patients before starting chemotherapy or radiotherapy. The study population consisted of 27 patients with various solid tumors (mean age +/- SD; 5.5 +/- 5.1 years, range; 0.2-14.5 years), 5 patients with acute leukemia (10.3 +/- 6.1, 1.3-17.0 years), and 26 healthy controls (11.6 +/- 4.0, 4.4-22.2 years). Although the age distributions were different, the mean Mf values of the hprt and the TCR loci were comparable among these three groups. On an individual basis taking the age factor into consideration, the hprt-Mfs of 3 patients with solid tumors, i.e., two patients with Hodgkin's disease and one patient with Askin tumor, were found to be well above the 95% confidence limit. There were no patients with a TCR-Mf exceeding the 95% confidence limit. These data suggest the possibility that some patients with solid tumors may be predisposed to mutational susceptibility before treatment. The assay of the hprt-Mf appears more sensitive than the TCR-Mf assay in distinguishing these patients.
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PMID:Evaluation of mutant frequencies at the hprt and the T-cell receptor loci in pediatric cancer patients before treatment. 954 58

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


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