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

Cell adhesion molecules (CAMs) of the immunoglobulin supergene family may play important roles in tumorigenesis and the development of metastatic disease. In a variety of human malignancies, tumor progression has been observed to be associated with changes in CAM expression. An early event in colorectal tumorigenesis appears to be the down regulation of a normally expressed CAM, DCC. Over-expression of a second CAM, carcinoembryonic antigen, is associated with colorectal tumors which have a high risk for metastasis development. Several tumors, including Wilms tumors and neuroblastoma, have been found to express a developmentally regulated form of NCAM which inhibits a variety of cell-cell interactions. Malignant cells not only show aberrations in the expression of their CAMS and thus their normal cell-cell interactions, but establish new adhesive interactions. The development of metastatic potential in cutaneous melanoma is associated with the de novo expression of two CAMs, one of which is ICAM-1, a molecule mediating adhesion between the tumor cells and leukocytes.
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PMID:Cell adhesion molecules of the immunoglobulin supergene family and their role in malignant transformation and progression to metastatic disease. 168 May 75

The expression of the disialoganglioside GD2 was analyzed in 67 solid tumors and normal tissues from children by using the GD2-specific murine monoclonal antibody 3A7 and the indirect immunoperoxidase method. GD2 was expressed in all of 28 neuroblastomas and was most abundant in stroma-poor tumors. In differentiating stroma-rich neuroblastomas, neuroblastic clusters, neurofibrils, and most ganglion-like cells were positive, whereas Schwann's-cell stroma did not express GD2. In ganglioneuromas, only a few ganglion-like cells showed GD2, whereas all other structures were negative. Scattered foci of ganglioside GD2 also were found in some non-neuronal tumors, such as rhabdomyosarcomas and osteosarcomas, but not in lymphomas, Askin tumors, or most Wilms' tumors. The monoclonal antibody 3A7 is a useful aid in the immunohistochemical diagnosis of neuroblastoma. In addition, the intense cell surface staining of neuroblastoma cells by this reagent makes it potentially useful for detecting residual neuroblastoma in bone marrow samples and lymph node biopsies.
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PMID:Cell-surface ganglioside GD2 in the immunohistochemical detection and differential diagnosis of neuroblastoma. 171 42

Ultrasound-guided percutaneous needle biopsy proved to be a reliable and safe method to obtain material for histopathological and immunohistochemical diagnosis prior to treatment in childhood malignancies. A principal tumour identification could be obtained by a combined morphological and phenotypic examination of 38 small-sized tumour biopsy specimens using a fairly limited panel of immunological reagents, including antibodies to leucocyte common antigen (CD 45), certain B- and T-cell markers, various intermediate filaments (cytokeratin, desmin and vimentin), and neuroblastoma cells (UJ 167.11, A2B5, and UJ 13A; the latter recognizes NCAM). Five undifferentiated neuroblastomas were all positive with the neuroblastoma antibodies but negative for the other markers, including vimentin. The negative reactivity for desmin and vimentin was the major immunohistochemical distinction between neuroblastomas and rhabdomyosarcomas. In addition, limited reactivity with the neuroblastoma antibodies was seen in blastematous parts of Wilms' tumour, duct-like structures in a hepatoblastoma, and in tumour cells in a few undifferentiated myelo- and lympho-proliferative lesions. This study shows the importance of a combined evaluation of morphology and the pattern of immunoreactivity employing multiple markers.
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PMID:Immunohistochemistry on needle biopsies of childhood malignancies. 172 20

High-dose thiotepa was given as a single agent at a total dose of 1125 mg/m2 with autologous bone marrow rescue to nine patients with recurrent/refractory/poor risk pediatric malignancies (primitive neuroepithelial tumor (PNET), two; neuroblastoma, one; Wilms' tumor, one; osteosarcoma, one; Ewing's sarcoma one, Hodgkin's disease one, high-grade glioma, two). The response rate in these heavily pretreated patients was 71% (five out of seven evaluable patients) including two complete responses (Wilms', glioma), three partial responses (osteosarcoma, Ewing's sarcoma, Hodgkin's disease), and two with stable disease (PNET, glioma). The median duration of response was 2.5 months. The extramedullary toxicity was acceptable with symptoms mainly of skin and gastrointestinal tract. The data indicate that high-dose thiotepa is effective in several types of recurrent pediatric solid tumors, and merits further evaluation in combination regimens.
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PMID:High-dose thiotepa with autologous bone marrow rescue in pediatric solid tumors. 176 72

Between 1987 and 1991, 248 long-term survivors of childhood cancer have been studied at the Oncology Unit of the Children's Hospital of Buenos Aires. The tumors were diagnosed between 1965 and 1986 as, retinoblastoma: 65, lymphoma: 57, nephroblastoma: 36, neuroblastoma: 25, germ-cell tumors: 18, sarcoma: 19, bone tumors: 7, lymphoepithelioma of cavum: 4, histiocytosis X: 9, others: 8. The treatment consisted of, surgery: 25, surgery+chemotherapy: 40, surgery+radiotherapy: 8, chemotherapy: 23, chemotherapy+radiotherapy: 42, and surgery+chemotherapy+radiotherapy: 110. There are alive without evidence of cancer disease 234 survivors between 5 and 25 years after diagnosis. Severe organic disabilities were observed in 181 survivors and moderate in 142. Thirteen patients died because of second malignant neoplasia and 1 patient with lung metastasis 9 years after diagnosis of nephroblastoma. In 180 survivors the data of instruction was available. Seventy participate in sports and 13 in artistic activities. Eight survivors are university graduates and 24 are employees. Five young women were mothers and one young man was a father. The meaning of concept of "cure" is discussed from the point of view of the physicians and the survivors. In order to detect deleterious late effects of cancer and their treatment the follow-up must be continuous.
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PMID:[The concept of cure in children with cancer]. 182 19

A total of 2259 children with solid malignant tumors were treated at St. Jude Children's Research Hospital between the years 1962 and 1987. Of these, 112 (5%) developed spinal epidural metastasis with spinal cord compression during the course of their disease process. Metastatic epidural spinal cord compression was caused most commonly by Ewing's sarcoma and neuroblastoma, followed by osteogenic sarcoma, rhabdomyosarcoma, Hodgkin's disease, soft-tissue sarcoma, germ-cell tumor, Wilm's tumor, and (rarely) hepatoma. There was no significant difference in outcome between patients with small-cell tumors (neuroblastoma, Hodgkin's disease, and germ-cell tumors) who received only chemotherapy and/or radiation therapy and the patients with similar lesions who received a decompressive laminectomy alone or prior to chemotherapy and/or radiation therapy. Patients with spinal cord compression from metastatic sarcoma (Ewing's sarcoma, soft-tissue sarcoma, osteogenic sarcoma, and rhabdomyosarcoma) showed a significant improvement with decompressive laminectomy alone or before medical therapy, compared to those who received radiation therapy and/or chemotherapy without posterior decompression. Pediatric tumors invade the spinal canal via the neural foramen, compressing the spinal cord in a circumferential manner, allowing decompressive laminectomy (posterior approach) to be an effective surgical approach. Sixty-six percent of children who had no evidence of motor or sensory function below the level of the compression became ambulatory after surgical decompression and medical treatment, regardless of tumor type.
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PMID:Pediatric spinal epidural metastases. 184 14

Constitutional chromosome abnormalities that may predispose a group of individuals to develop certain neoplasms have been reported for some human solid tumors. Deletions of 13q in retinoblastoma, 11p in Wilms' tumor, 1p in neuroblastoma, 3p in renal cell carcinoma, 5q in colorectal carcinoma and 22q in meningioma are examples of such anomalies. In breast carcinoma, a specific cytogenetic defect has not been conclusively identified. We have studied Phytohemagglutinin-stimulated lymphocytes of 76 breast cancer patients, 68 predisposed family members, 40 controls, and 30 additional controls with lung cancer to determine whether nonrandom chromosome defects are present. From each sample 100, G-or Q-banded metaphase spreads were studied for rearrangements. A marked clustering of alterations in the long arm of chromosome no. 1 (q11-22) was seen in breast cancer patients and in some predisposed family members. Alterations in 1q were present in 1% to 3% of metaphases, and included translocations to chromosomes 3, 6, 7, 9, 12, 15, 17, 18, 21 and the X; deletion of 1q, or pericentric inversion. Twelve out of 62 (19.3%) familial cases, 3 out of 14 (21.4%) sporadic cases, 9 out of 68 (13.2%) predisposed cases and 2 out of 40 (5%) control cases showed 1q alterations. None of the 30 lung cancer patients showed chromosome 1 anomaly in this region. This is consistent with the reports on primary breast tumor tissues, cell lines and pleural effusions where 1q defects have been reported. We conclude that chromosome 1q rearrangement might be one of the primary lesions specifically associated with the development of breast cancer.
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PMID:Chromosome anomalies in human breast cancer: evidence for specific involvement of 1q region in lymphocyte cultures. 188 38

Magnetic resonance imaging (MRI) was performed in 126 children with malignant solid tumor between April 1984 and December 1990. The criteria of tumor visualization, localization, staging, prediction of kidney preserving and monitoring treatment were compared by MRI and CT for 47 children with neuroblastoma, Wilms' tumor, hepatoblastoma, rhabdomyosarcoma and teratoma, MRI and CT were viewed together and an assessment was made as to whether the studies yielded equivalent information or whether one study was superior to the other. 1) The tumor were better visualized in 47% cases by MRI than CT. 2) MRI was superior to CT in 43% cases in evaluating the local spread of tumor. 3) There was little difference between MRI and CT in identification of lymph node metastases. 4) Without requiring the injection of intravenous contrast agents, MRI accurately defined displacement, invasion of renal vessels by neuroblastoma. MRI was excellent in prediction of kidney preserving. 5) MRI was useful to detect bone marrow metastases in neuroblastoma. The best imaging plane for a demonstration of bone marrow involvement was coronal in lower limbs.
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PMID:[The role of magnetic resonance imaging for treatment in children with malignant solid tumor]. 194 73

In order to investigate the number, types, severity and the various treatments of spinal deformity among children under 10 years old, a retrospective survey of registered patients in the scoliosis clinic at the National Taiwan University Hospital was performed. Between August 1982 and December 1988, there were 41 children who had scoliosis with a Cobb angle larger than 10 degrees and the onset was before 10 years of age. This number accounted for 3.8% of all scoliotic patients during the same time period. Of these children, 19 had idiopathic scoliosis (46.3%), 7 with infantile and 12 with juvenile; the other 19 were due to congenital, and the remaining 3 were postradiation, a resection of Wilms' tumor in 2 and neuroblastoma in 1. In the congenital group, hemivertebra (13 patients) outnumbered other causes. Twenty three patients (56%) underwent surgical correction, the rest were either under regular observation (9 patients), bracing (7 patients) or electrical stimulation (2 patients). The average preoperative Cobb angle in the operated groups was much larger, being 67.6 degrees in the infantile; 52.4 degrees in the juvenile; 57 degrees in the congenital; and 62 degrees in the postradiation. For those without an operation, the angles were smaller than 30 degrees. The indications for surgery were that the curvature was in progression, which could not be controlled by conservative means, or that in some congenital cases, the curve had the potential tendency to exacerbate. From the present study, the percentage of scoliosis under 10 years of age was far less than the adolescent group in our clinic.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Spinal deformities among children under 10 years old: a clinical analysis of 41 cases. 198 37

Autologous bone marrow transplantation (ABMT) allows delivery of intensive, marrow-ablative chemotherapy or chemoradiotherapy to children with high-risk solid tumors. Results from several studies of neuroblastoma suggest that outcome is improved by ABMT; however, relapses can occur months to years after complete clinical remission. Other high-risk tumors including peripheral neuroepithelioma, Ewing's sarcoma, rhabdomyosarcoma, Wilms' tumor, and brain tumors also appear to be responsive to intensive marrow-ablative therapy, although few studies have been reported. For tumors that can metastasize to marrow, a sensitive method is necessary for detecting tumor cell contamination. Immunocytologic analysis with monoclonal antibodies can identify one neuroblastoma cell per 10(5) normal marrow cells; this method also is applicable to other tumors with appropriate antibodies. Ex vivo removal (purging) of tumor cells decreases the probability of infusing tumorigenic cells with the ABMT. There is considerable experience in tumor detection and purging for neuroblastoma, but little has been done for other childhood solid tumors. Future investigations of ABMT will aim to further increase disease-free survival by intensifying induction and marrow-ablative regimens and by developing therapies to be given after ABMT that are directed at minimal residual disease. As pilot investigations mature, the efficacy of ABMT and conventional chemotherapy will be compared in multi-institution randomized studies.
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PMID:Treatment of high-risk solid tumors of childhood with intensive therapy and autologous bone marrow transplantation. 200 84


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