Browsing by Subject "Neuroblastoma"

Now showing 1 - 5 of 5
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    1p36 deletion results in a decrease in glycosaminoglycans which is associated with aggressiveness in neuroblastic tumors
    (Universidad de Murcia. Departamento de Biología Celular e Histología, 2018) Tadeo, Irene; Gamero Sandemetrio, Esther; Berbegall, Ana P.; Navarro, Samuel; Cañete, Adela; Noguera, Rosa
    Despite our deep understanding of neuroblastic tumors, some patients still suffer treatment failure, so pre-treatment risk stratification still requires improvement and the search for new therapeutic targets must continue. Here we correlated prognostic clinical and biological features of neuroblastic tumors with the density of extracellular matrix glycosaminoglycans (the main components of the extracellular matrix ‘ground substance’), in nearly 400 primary samples. We also studied the relationship between the density of extracellular matrix glycosaminoglycans and the expression of B3GALT6, an enzyme required for their synthesis. We associated a decrease in glycosaminoglycans with neuroblastomas that were histopathologically poorly-differentiated or undifferentiated, as well as with metastatic disease, and 1p36 deleted tumors. This decrease in glycosaminoglycans was also related to abnormal nuclear B3GALT6 expression in neuroblastic cells. These findings point towards the importance of the ground substance in the aggressiveness of neuroblastic tumors, which should therefore be considered when developing novel therapies for treating neuroblastomas.
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    Apoptosis in peripheral neuroblastic tumors. Immunohistochemical expression of bcl-2 and p53 is related to DNA fragmentation
    (Murcia: F. Hernández, 2007) Mejía, C.; Navarro, S.; Llombart-Bosch, A.
    We examined 111 cases of neuroblastoma (NB), searching for how NB relates to apoptotic control and other prognostic factors. Immunohistochemistry using avidin-biotin-peroxidase was carried out for bcl-2 and p53 proteins. Apoptosis was analyzed by in situ detection of chromosomal breakdown. DNA ladders were detected by electrophoresis and amplification of MYCN was carried out by PCR and Southern blot. Statistical analyses were performed with Pearson’s c2 and Kruskal-Wallis tests and Cox’s regression. We found expression of bcl-2 protein mainly in cases of neuroblastoma without differentiation and in stages 3 and 4. Expression of p53 protein showed a correlation with bcl-2 and the apoptotic phenomenon; apoptosis was found mainly in favorable cases. Multivariate analysis showed bcl-2 protein expression to be the most independent risk factor. The study of apoptosis could be important for the design of therapies to treat neuroblastoma.
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    Comparative study of MLPA-FISH to determine DNA copy number alterations in neuroblastic tumors
    (Murcia: F. Hernández, 2011) Villamón, E.; Piqueras, M.; Berbegall, A.P.; Tadeo, I.; Castel, V.; Navarro, S.; Noguera, R.
    Neuroblastoma tumor cells show complex combinations of genetic aberrations, and to date many different methods have been used for their detection. To apply genome-wide techniques, such as Multiplex Ligation–dependent Probe Amplification (MLPA), in routine diagnosis their validation is appropriate and necessary. DNA copy number alterations in 129 cases of neuroblastic tumors were detected using MPLA, and the results validated by Fluorescence In Situ Hybridization (FISH) (MYCN gene, 1p36, 11q and 17q). Kappa index values showed very good concordance between the two techniques in detecting homogeneous MYCN amplification (1); 11q deletion (0.908) and 17q gain (0.922). The validation results showed that MLPA is a highly efficient technique for diagnosis based on the genetic aberrations in relevant regions in neuroblastoma, showing a high concordance with FISH.
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    lmmunohistological signposts in central nervous system tumours with neuronal differentiation
    (Murcia : F. Hernández, 1991) Cruz-Sánchez, F. F.; Rossi, M.L.; Rodríguez-Prados, S.; Cusi, V.; Coakham, H.B.
    25 neuronal tumours with a panel of antibodies were studied and it was found that vimentin was present in 15 tumours. It was also found in a few cells within rosettes. PGP 9.5 showed a somatic pattern of staining with nuclear and perinuclear positivity in 23. Neurofilament reactivity was found in 14. Retina1 S-antigen was detected only in one medulloblastoma, 314 pineal tumours and 212 retinoblastomas. Reactivity. for synaptophysin was present in 215 medulloblastomas, 3/10 neuroblastomas and 212 retinoblastomas. GFAP was demonstrated in scattered tumour cells in 415 medulloblastomas. Two of these were the only tumours featuring bipolar differentiation whilst it was unipolar in the remainder. The significance of these findings in relation to the ontogeny of these tumours is discussed.
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    Neuroblastoma. A study of the clinicopathological features influencing prognosis based on the analysis of 54 cases
    (Murcia : F. Hernández, 1994) Valera-Durán, J.; Böhm, N.; Díaz-Flores, Lucio; Ramon y Cajal-Junquera, S.; Toro Rojas, M.; Valera-Nuñez, R.
    The retrospective analysis of 54 cases of neuroblastoma taken from the files of the Department of Pathology, University of Santiago Hospital, Spain, and the Ludwig-Aschoff Institute of Pathology, University of Freiburg, Germany confirmed the validity and significance of various clinical and histopathological features when trying to establish the prognosis and the proper therapeutic approach in a given case of neuroblastoma. When the age of the patients was compared to survival it was shown that all but three of the patients older than 2 years of age had died from tumor within ten months. In contrast, there was a 37.5% five-year survival rate among patients who were 24 months of age or younger at the time of diagnosis and treatment. The primary tulnor was located in the adrenal gland in 27 cases (50%), in 9 cases (17%) the tumor was retroperitoneal but extra-adrenal, and in the remaining 18 patients (33%) the tumor arose from the paravertebral sympathetic ganglia. Adrenal primaries behaved in an extremely aggresive manner as all but three patients with tilmors at this location were dead within 18 months. Retroperitoneal extra-adrenal neuroblastomas followed an almost equally poor outcome with only one five-year survivor ( 1 1 %). In contrast, 49% of the patients with paravertebral neuroblastoma had survived five years and a further 33% were alive with shorter follow-up. According to histological criteria, there were 6 grade I turnors, 15 grade I1 and 33 grade 111 tumors in our series. All grade I tumors were clinical stage 1 at diagnosis and all are alive 2 to 3 112 years later. Grade I1 tumors were clinical stage 2, 3 or 4 and showed a 46% five-year survival. With the exception of three patients with paravertebral tumors, all patients with grade 111 neuroblastoma were clinical stage 3 or 4 when initially Offprint requests to: Dr. Juan Varela-Duran, Departamento de Anatomia Patologica, Hospital General de Galicia, C/ Galeras s/n, 15702 Santiago de Cornpostela, Spain seen and all were dead from tumor within ten months, with a five-year survival of 9%. It is concluded that the age at diagnosis, location of the primary tumor and histological differentiation, all of which are interrelated, are the most reliable clinicopathological features affecting prognosis and therapy in neuroblastoma.