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)

Identification of growth factors and receptors in mesenchymal tumors may be crucial to understanding of growth regulation in sarcomas. During an immunohistochemical study of the expression of growth factors and receptors in human soft tissue tumors (STT), only 1 antisera capable of working in paraffin-embedded tissue was noted. A detailed study of 141 STT was undertaken to determine the frequency of expression of nerve growth factor receptor (NGF-R), its specificity and sensitivity for neural tumors, and the effect of fixation on detection. In normal mesenchymal tissue, only nerve sheath and perivascular staining was seen. No immunoreactivity was seen in many tumors including rhabdomyosarcoma, angiosarcoma, liposarcoma, Ewing's sarcoma, and alveolar soft part sarcoma. Less than 15% of tumors of smooth muscle, fibrous, or fibrohistiocytic origin showed immunoreactivity, usually focal. In contrast, a high frequency of immunoreactivity was noted in tumors of neural origin (74%). This included granular cell tumors (100%), Schwannoma/neurofibroma (91%), malignant Schwannoma (78%), neuroblastoma/neuroepithelioma (60%), and paraganglioma (57%). A high rate of reactivity was also seen in synovial sarcomas (80%), undifferentiated sarcomas (60%), and hemangiopericytomas (43%), suggesting a potential relationship to the neural phenotype. Among the neural tumors, Bouin's fixation was superior to formalin, suggesting that immunoreactivity for NGF-R is affected by fixation. This antibody may be a useful adjunct marker diagnostically.
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PMID:Expression of nerve growth factor receptor in paraffin-embedded soft tissue tumors. 245 20

The pharmacokinetics of alkylating activity were studied in 17 children treated i.v. with ifosfamide (IF) at 3 g/m2 as a 1-h infusion for 2 consecutive days every 3 weeks, with mesna as a uroprotector. Two patients were treated for a newly diagnosed rhabdomyosarcoma according to the current SIOP (International Society of Pediatric Oncology) protocol. The other 15 patients were treated in a phase II study and presented with one of the following malignancies in relapse: neuroblastoma (7), osteosarcoma (3), soft tissue sarcoma (2), Wilms' tumor (1), non-Hodgkin's lymphoma (1), and acute lymphoblastic leukemia (1). Plasma alkylating activity levels determined by using 4(4'-nitro-benzyl)-pyridine showed considerable inter-individual and intercyclic variations and decreased biphasically, with mean alpha and beta half-lives of 60 min and 6-7 h, respectively. Probably as a result of liver mixed-function oxidase induction, on the 2nd day of treatment the terminal half-lives were shorter, the plasma exposures were lower, and the mean plasma clearances were higher. Renal excretion was almost complete after 24 h, accounting for a mean of 19% of the injected dose. The CSF alkylating activity levels, obtained in four children, were always lower than the plasma levels and ranged from 8 to 51 micrograms/ml, with a mean CSF/plasma ratio of 0.53 +/- 0.23 during the first 12 h. We conclude that IF alkylating activity was biphasically cleared from the plasma, with significant interindividual and intercyclic variability, that the renal contribution to the clearance was low, and that high levels of CSF alkylating activity could possibly contribute to the CNS toxic side effects observed in pediatric patients treated with high-dose IF/mesna.
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PMID:Alkylating activity in serum, urine, and CSF following high-dose ifosfamide in children. 250 56

Ifosfamide/mesna was given to 97 patients who had malignant solid tumors diagnosed before they were 21 years of age. Patients received 1.6 g/m2 ifosfamide daily x 5, given i.v. over 15 min, followed by 400 mg/m2 i.v. mesna at 15 min and 4 and 6 h after ifosfamide. Responses were noted in patients with osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma and other soft-tissue sarcomas, rhabdoid tumor, neuroblastoma, Wilms' tumor, primitive neuroectodermal tumor, retinoblastoma, germ-cell tumors, and B-cell lymphoma. Toxicity included mild to moderate nausea and vomiting, transient, reversible myelosuppression, transient elevations of serum blood urea nitrogen (BUN) and creatinine and liver enzymes, infections, and self-limiting neurotoxicity characterized by changes in mental status, motor dysfunction, cranial nerve palsy, cerebellar dysfunction, and seizures. Neurotoxic symptoms were generally seen in patients who had previously received cisplatin. Ifosfamide is an important alkylating agent that should be combined with other agents in phase II and III trials. Alternate dose schedules should also be investigated.
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PMID:Ifosfamide in pediatric malignant solid tumors. 250 57

The presence of neonatal (cord) lymphokine-activated killer (LAK) cell activity toward natural killer cell resistant Raji and Daudi cell lines has recently been reported from our laboratory. We investigated the future therapeutic use of LAK adoptive immunotherapy by examining LAK in vitro cytotoxicity from both neonatal and adult mononuclear cells against solid tumor cell lines of relevance to pediatric oncology: SH-SY5Y (neuroblastoma), SK-NM-C (neuroblastoma-neuroepithelioma), NEP-1 (Wilms' tumor), SK-ES-1 (Ewing's sarcoma), and A-204 (rhabdomyosarcoma). Cord and adult mononuclear cells were activated by recombinant IL-2 (100 mu/ml) for 5-7 days and added in an effector:target ratio of 40:1 to 51Cr-labeled target cells. Specific cell lysis was determined after a 4-h incubation. There was a significantly high level of cord and adult LAK cytotoxicity against Wilms' (76.4 +/- 9.8 versus 77.3 +/- 6.8%) and Ewing's (84.2 +/- 5.5 versus 71.1 +/- 6.5%) cell lines and significant but moderate LAK activity against neuroepithelioma (52.0 +/- 6.6 versus 55.4 +/- 4.5%) and rhabdomyosarcoma (46.6 +/- 5.7 versus 43.9 +/- 5.2%) cell lines. There was no difference between cord and adult LAK activity toward these targets. However, a differential response toward the more classical neuroblastoma cell line, SH-SY5Y, was noted with significantly more LAK cytotoxicity from cord mononuclear cells than adult mononuclear cells (51.2 +/- 6.9 versus 28.5 +/- 8.2%) (p less than or equal to 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Lymphokine-activated killer cytotoxicity in neonatal mononuclear cells: in vitro responses to tumor cell lines from pediatric solid tumors. 253 88

Recently, great interest has been shown in the histological identification of small cell tumours of childhood--nephroblastoma (Wilms' tumour), neuroblastoma, rhabdomyosarcoma and Ewing's sarcoma--using immunohistochemical methods. However, several antigens operationally specific for leucocyte typing in blood and marrow are also expressed on cells of epithelial and neural origin. We undertook phenotypic characterization of 17 non-haemopoietic small cell tumours of childhood using a panel of 30 monoclonal antibodies to leucocyte, epithelial and cytoskeletal antigens using a sensitive alkaline phosphatase-anti-alkaline phosphatase technique on cryostat sections of fresh tumour. Our results demonstrated frequent expression of the leucocyte-associated antigens CD10 (CALLA), CD9 (p24) and CDw32 (FcRII) in these small cell tumours and occasional expression of MHC class II (HLA-DR) and HNK-1 antigens. However, the leucocyte-associated antigens CD45 (leucocyte common), CD22 (pan B-cell), CD11b (C3bi receptor), CD15 (Lewisx) or CDw42 (platelet gp Ib) were not detected on any tumour. Aberrant expression of desmin, neurofilament and UJ13A antigen was found in nephroblastoma and of epithelial-associated markers (CIBr17 and 43-9F) in neuroblastoma. Our results also demonstrated broad reactivity in frozen section with two monoclonal antibodies specific for melanoma (NKI/C-3) or epithelial cells (OM-1) in paraffin sections. Hence, it is necessary to include monoclonal antibodies to CD45 and pan-epithelial antigens, e.g. LP34 (cytokeratin) or HEA125 for the precise immunohistochemical identification of small round cell malignancies of childhood.
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PMID:Phenotypic characterization of non-haemopoietic small cell tumours of childhood with monoclonal antibodies to leucocytes, epithelial cells and cytoskeletal proteins. 254

Controversy exists regarding the most appropriate treatment for the rare adult patient who develops a so-called pediatric cancer. We have reviewed our 20-year experience with these patients and analyzed their outcome. A total of 299 patients with rhabdomyosarcoma (106), Wilms' tumor (97), and neuroblastoma (96) were evaluated and treated at Stanford University Medical Center between January 1967 and December 1987. Only 26 of these patients (8.7%) were diagnosed during "adulthood"; their age range was 18-67 years, median 23 years. Wilms' tumor; Five patients presented with Wilms' tumor at age greater than or equal to 18 years; four had unfavorable histology. All underwent multimodality therapy; however, only two have survived, one currently disease-free and one with disease. Neuroblastoma: Five patients presented with neuroblastoma at age greater than or equal to 18 years. Four underwent attempted surgical resection, post-operative irradiation (RT), and chemotherapy (CT); the other received no adjuvant CT. Only two of the five patients survive, both with disease. Rhabdomyosarcoma: Of the 16 adults (greater than or equal to 21 years) with rhabdomyosarcoma, 14 (87%) had advanced Intergroup Rhabdomyosarcoma Study-group disease (eight Group III, six Group IV). All 16 underwent aggressive multimodality therapy. At 10 months-16 years follow-up, only five patients survive, four of whom are apparently cured of their tumor. Neither histologic subtype nor site of presentation were of prognostic value. This series demonstrates that adults with Wilms' tumor, neuroblastoma, or rhabdomyosarcoma have a worse prognosis than do children with the same diagnosis. Possible explanations for this disparity in outcome include different tumor biology, less tolerance for treatment, and different natural history among adults relative to children.
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PMID:Treatment results among adults with childhood tumors: a 20-year experience. 255 Mar 96

Nonchromaffin paraganglioma (NCP), also called glomus body tumor or chemodectoma, is rarely found in the orbit. The behavior of orbital nonchromaffin paraganglioma may potentially be more aggressive than in other head and neck locations. Diagnosis depends on electron microscopic demonstration of membrane-bound neurosecretory granules. Results of histopathologic study show a well-circumscribed lesion without a true capsule with alveolar or organoid arrangements of epithelioid cells within a reticulin framework with thin-walled blood vessels. Cells are polygonal with round or oval nuclei containing rare mitotic figures and pale-staining cytoplasm. Differential diagnosis includes alveolar soft-part sarcoma, alveolar rhabdomyosarcoma, neuroblastoma, carcinoid, and granular cell tumor. Of 29 previously reported cases of orbital NCP, 16 have been reclassified as alveolar soft-part sarcoma. The authors report a patient with an electron microscopically established orbital NCP, with the history of a contralateral glomus jugulare tumor irradiated 14 years previously.
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PMID:Orbital nonchromaffin paraganglioma. A case report and review of the literature. 255 86

Congenital alveolar rhabdomyosarcoma in a newborn was reported. She had multiple subcutaneous tumors and looked like a blueberry muffin baby. From the clinical signs and the histological pictures, a diagnosis of neuroblastoma was considered. Laboratory data showed no abnormalities. Electron microscopic examination revealed myofilament and incipient stria formation. On the basis of these data, a final diagnosis of congenital alveolar rhabdomyosarcoma associated with multiple skin metastases was made.
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PMID:Congenital alveolar rhabdomyosarcoma presenting as a blueberry muffin baby. 260 Feb 81

531 cases of malignant neoplasms and potentially malignant hyperplasia in children and adolescents diagnosed in the Department of Pathomorphology, Institute of Mother and Child between 1978/79 and 1983 are presented. Only patients treated in this hospital were included into the study. The most common diagnoses were neuroblastoma (68 cases), rhabdomyosarcoma (62 cases), nephroblastoma (56 cases), osteogenic sarcoma (47 cases), non-Hodgkin lymphoma (41 cases). Besides neoplasms most typical for childhood and adolescence the rare less typical cases were also seen.
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PMID:[Malignant neoplasms and potentially malignant hyperplasia in children and adolescents from material at the Department of Pathomorphology, Institute of Mother and Child during 1978/79-1983]. 263 66

The majority of lesions in the neck of children will prove to be benign and of congenital origin arising from such structures as the thyroglossal duct and the branchial clefts in addition to hemangiomas and lymphangiomas. However, it is essential that lymphadenopathy be proven infectious and any asymptomatic lymph node enlargement must be considered Hodgkin's disease until proven otherwise. The occasional neuroblastoma or rhabdomyosarcoma presenting in the neck can usually be identified as solid by sonography, leading to early investigation and biopsy. Because of the abundance of important structures that course through the neck, surgery should be conducted in an operating suite with sophisticated, modern anesthetic techniques and with a surgeon experienced in dealing with the full array of lesions that occur.
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PMID:Common lumps and bumps of the head and neck in infants and children. 266 78


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