Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

All cases diagnosed in Finland as non-Hodgkin's lymphoma (NHL), Hodgkin's disease or histiocytosis X in children younger than 15 years in 1953 to 1973, according to the Finnish Cancer Registry, were reexamined histologically. Only 55% of the cases originally diagnosed as NHL were regarded as such at reexamination. The others were mainly malignant nonlymphatic tumors such as neuroblastoma and different kinds of sarcomas. Seventy-two NHLs were diagnosed in 50 boys and 22 girls. The corrected age-specific incidence rate was 0.32/10(5). The most common histologic types were Burkitt's lymphoma (BL) (30 cases), lymphoblastic lymphoma (LBL) (26), large cell lymphomas (LCL) (six), and non-Burkitt's lymphoma (n-BL) (three). There were marked differences between BL and LBL in the course of the disease: BL was extranodal in 83%, LBL only in 4% (mediastinum was regarded as nodal); BL showed initial abdominal or pelvic involvement in 60% whereas LBL showed none; BL had initial mediastinal involvement in 7%, and LBL had it in 62%; all patients with LBL died whereas 23% of those with BL survived. Other types of NHL resembled BL in their course of disease. Patients with initial tonsillary involvement appeared to have the best prognosis and patients with mediastinal involvement the poorest. The importance of accurate histologic classification is emphasized. It appears to be most important to differentiate LBL from other types of NHL.
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PMID:Non-Hodgkin's lymphomas in childhood. A clinicopathologic and epidemiologic study in Finland. 349 36

Two cases of fatal interstitial pneumonitis developing after an autologous bone marrow transplantation are described. In both cases the autopsy revealed diffuse malignant pulmonary involvement. The first case involved a 4-year-old boy who had a Burkitt's lymphoma; the second case involved a 4-year-old girl with a neuroblastoma. The authors postulate that in these cases, the clinical picture may have been related to reinfusion of malignant cells.
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PMID:Metastatic interstitial pneumonitis after autologous bone marrow transplantation. A consequence of reinjection of malignant cells? 353 Apr 32

The oncogene of the HL-60 human promyelocytic leukemia cell line has been passed serially through NIH/3T3 mouse fibroblasts. Oncogene-specific probes prepared from the resulting tertiary transfectants by molecular cloning have been used to show that loss of the transfected oncogene from NIH/3T3 cells correlates with reversion to nontransformed morphology. Analysis of cells transfected by the oncogenes of other tumors and tumor cell lines indicates that the transforming gene of the HL-60 leukemia cell line is closely related to oncogenes of a Burkitt's lymphoma, an acute myelogenous leukemia, an adenocarcinoma of the colon, a neuroblastoma, and two sarcomas. This oncogene is distantly related to the viral oncogenes of Kirsten and Harvey sarcoma viruses. It has been termed N-ras. The active N-ras oncogene coexists with altered versions of the myc oncogene in the HL-60 and AW Ramos human tumors. This suggests a multistep mechanism involving both ras and myc genes in the creation of these tumors.
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PMID:The HL-60 transforming sequence: a ras oncogene coexisting with altered myc genes in hematopoietic tumors. 668 94

One of the first oncogenes identified from human tumors was c-myc, which is frequently activated in Burkitt's lymphomas due to chromosomal translocations. Subsequently, members of the myc oncogene family were found to be amplified in neuroblastoma and small-cell lung cancer. In normal cells, Myc activity has been shown to be both necessary and sufficient for resting cells to enter the cell cycle. Interestingly, it appears that Myc not only drives the cell cycle, but also induces cell death by apoptosis in certain situations. Myc contains a transcriptional activation domain and a basic helix-loop-helix-leucine zipper DNA-binding and dimerization domain. As a heterodimer with a structurally related protein, Max, Myc can bind DNA in a sequence-specific manner. These results suggest that the Myc/Max heterodimer functions as a transcriptional activator of genes that are critical for the regulation of cell growth.
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PMID:Myc protein: partners and antagonists. 794 8

Three cases of secondary (therapy-related) hematologic malignant conditions were identified among 95 children as old as 18 years of age; the cases were diagnosed between 1984 and 1990 and consisted of acute lymphoblastic leukemia, acute myeloid leukemia (AML), and myelodysplastic syndrome (MDSs). They constituted 10% of all new cases of AML and MDS seen at the University Hospitals of Cleveland during this time and were not related to congenital factors. The primary malignant conditions were malignant thoracopulmonary tumor (Askin tumor), neuroblastoma, and Burkitt's lymphoma. The secondary hematologic disorders all showed a prominent monocytic component: acute monocytic leukemia, MDSs evolving to acute myelomonocytic leukemia, and chronic myelomonocytic leukemia. The mean interval between treatment for the primary malignant condition and the onset of secondary disease was 36 months. All had received cyclophosphamide and an epipodophyllotoxin for the primary tumor; two were treated with radiation therapy. Cytogenetic abnormalities included del(5), del(13), t(1;6), and t(9;11)(p22[symbol:see text]3). The survival time after the onset of secondary disease was short.
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PMID:Myelodysplastic syndrome and acute myeloid leukemia after treatment for solid tumors of childhood. 837 35

We previously have reported on an experimental athymic mouse model in which regression of human Burkitt's lymphoma is induced by either coinjection with or intratumor inoculation of Epstein-Barr virus (EBV)-immortalized human B cells. In the current study, we were interested in determining whether the powerful antitumor effects of EBV-immortalized B cells could be effective against a variety of human tumors grown in athymic mice, including acute lymphocytic leukemia, malignant melanoma, acute promyelocytic leukemia, neuroblastoma, lung carcinoma, colon adenocarcinoma, Wilms tumor, Hodgkin's lymphoma, rhabdomyosarcoma and breast adenocarcinoma. We report here the results of experiments in nude mice that demonstrated the potent antitumor effect of EBV-immortalized B cells against human tumors derived from a variety of different tissues.
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PMID:Regression of experimental human leukemias and solid tumors induced by Epstein-Barr virus-immortalized B cells. 853 18

The total incidence of childhood cancer varies rather little between different regions of the world, with cumulative risk to age 15 nearly always in the range 1.0-2.5 per thousand. Acute lymphoblastic leukaemia, especially in early childhood, is most common in populations of high socio-economic status and is the most frequent childhood cancer in all industrialised countries. The risk of Burkitt's lymphoma is highest in tropical Africa and Papua New Guinea; it is strongly associated with Epstein-Barr virus infection and intense immune stimulation by malaria. Other lymphomas are also relatively common in developing countries. Non-heritable retinoblastoma has a higher incidence among less affluent populations, suggesting an association with poor living conditions and maybe an infectious aetiology. In contrast, the incidence of Wilms' tumour and Ewing's sarcoma varies largely on ethnic lines, indicating a strong role for genetic predisposition. Much of the variation in recorded incidence of brain tumours and neuroblastoma may be due to varying levels of case ascertainment. Recently the incidence of childhood Kaposi's sarcoma has risen substantially in parts of Africa severely affected by the AIDS epidemic.
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PMID:Geographic and ethnic variations in the incidence of childhood cancer. 903 26

Dural sinus thrombosis (DST) has been reported in association with cancer in both adults and children. We describe the seven patients seen with this complication in our centre between 1981 and 1995. Diagnosis was confirmed by either cerebral CT scanning, MRI or angiography. Median age was 13 years (range 8-15). Six patients were boys. Six children were being treated for non-Hodgkin lymphoma and one for neuroblastoma. Presenting symptoms were seizures and transient neurologic deficit, often preceded by headaches. The probable cause of DST was found in two cases. Tumour localisation in the central nervous system (CNS) probably caused DST in one patient who was treated for ki 1 lymphoma. Dehydration in combination with a poor general condition seemed to be the cause of DST in the patient with neuroblastoma. In five children with stage III or IV non-Hodgkin lymphoma (three lymphoblastic lymphoma; two Burkitt's lymphoma), etiology remained unknown. In these children, DST occurred early in the course of therapy. The median interval between start of chemotherapy and onset of symptoms was 19 days (range 8-40). No child had received L-asparaginase. Prognosis was favourable, with symptoms completely disappearing without therapy within 1 to 5 days. The incidence of DST in patients with advanced stage non-Hodgkin lymphoma during induction and consolidation was calculated to be below 3%. We conclude that DST is rarely diagnosed in children with cancer. Occurrence during the initial phase of therapy for non-Hodgkin lymphoma is associated with a benign prognosis.
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PMID:Dural sinus thrombosis in children with cancer. 1211 89

Solid malignant tumours (n = 263) excluding brain and spinal cord tumours in children up to 14 years of age were studied. Retinoblastoma (27%) constituted the largest group followed by Wilms' tumour (14.1%) and lymphoma (13.7%). Most patients (55%) were of less than 5 years age and maximum incidence of embryonal tumours was found in this age group; other tumours were more frequent in higher age. A male preponderance was noted (male to female ratio as 1.6:1). Amongst lymphoma, 61% were non-Hodgkin's lymphoma and rest were Hodgkin's disease; 2 cases of Burkitt's lymphoma were found. Other notable tumours encountered in the study were embryonal rhabdomyosarcoma (n = 14), hepatoblastoma (n = 9), neuroblastoma (n = 7), Ewing's sarcoma (n = 21), osteogenic sarcoma (n = 19) and germ cell tumours (n = 14).
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PMID:Pattern of solid malignant tumours in children--a ten-year study. 935 72

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


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