Nucleolar Organizer Region (nors) In Pseudocarcinomatous Hyperplasia And Squamous Cell Carcinoma Of The Oral Mucosa.

Counts of nucleolar regions (NORs) demonstrated by a silver staining technique in paraffin sections, have been used to distinguish benign from malignant lesions. AgNORs were studied in 24 biopsies from oral cavity (5 cases of normal oral mucosa, 5 of pseudocarcinomatous hyperplasia and 14 of squamous cell carcinoma, subdivided according to degree of differentiation: 5 grade 1, 5 grade 2 and 4 grade 3) to find whether they were helpful in distinguishing pseudocarcinomatous hyperplasia due to chronic parasitic infections from squamous cell carcinoma. Two methods of counting AgNORs were used: (A) a simpler one which counts nucleolar clusters (AgNU) and satellite AgNORs and the other (B) counting all individual AgNORs, including those within AgNUs. In both methods the lowest mean values were observed for grade 3 carcinoma, while the highest belonged to grade 2 carcinoma. The simpler method (A) was the most useful because AgNU counts showed significant difference when pseudocarcinomatous hyperplasia was compared with grade 1 and 2 carcinomas, which are the most difficult to discriminate from it. However the overlapping of values render the technique of limited use in individual cases.113169369

Morphometry And Histology Of Gonads From 13 Children With Dysgenetic Male Pseudohermaphroditism

Background. - Dysgenetic male pseudohermaphroditism (DMP) is a sexual differentiation disorder characterized by bilateral dysgenetic testes, persistent müllerian structures, and cryptorchidism in individuals with a 46,XY karyotype. However, the histologic criteria for the diagnosis of DMP are poorly established. Objective. - To determine gonadal histology in children with DMP. Patients and Methods. - Between 1996 and 1998, 13 patients with DMP were evaluated on our service. The clinical diagnosis of DMP was based on a 46,XY karyotype, sex ambiguity, high levels of follicle-stimulating hormone and low levels Of antimüllerian hormone, a decreased testosterone response to human chorionic gonadotropin stimulation without accumulation of testosterone precursors, and the presence of müllerian structures. Molecular sequencing the HMGbox region of the SRY gene did not reveal any mutations. Biopsies were performed for 22 of 26 gonads (patient age at the time of biopsy, 16 months to 10 years). Conventional microscopy was used to evaluate mean tubular diameter, tubular fertility index, and number of Sertoli cells per tubular profile. Results. - All 26 gonads were located outside of the labioscrotal folds. Their histologic features varied from only a reduction in tubular size to features of a streak gonad. Five of the 22 gonads grossly resembled a streak gonad. The mean tubular diameter was severely reduced (>30% reduction relative to the normal tubular diameter for the patient's age) in 4 gonads, markedly reduced (10%-30%) in 11 gonads, slightly reduced (<10%) in one gonad, and normal in one gonad. The tubular fertililty index, expressed as the percentage of tubular profiles containing germ cells, was severely reduced (<30% of normal values) in 9 gonads, markedly reduced (50%-30%) in 2 gonads, and normal in 6 gonads. The number of Sertoli cells per tubular profile was elevated in 16 gonads and normal in one gonad. Thin tubules surrounded by fibrous tissue were occasionally observed. Conclusion. - The histologic findings confirmed the clinical diagnosis of DMP in every patient in the present series. However, gonadal histology was variable, and careful morphometric evaluation may be necessary to establish the diagnosis.1255652656Robboy, S.J., Miller, T., Donahoe, P.K., Dysgenesis of testicular and streak gonads in the syndrome of mixed gonadal dysgenesis: Perspective derived from clinicopathologic analysis of twenty-one cases (1982) Hum Pathol, 13, pp. 700-716Troche, V., Hernandez, E., Neoplasia arising in dysgenetic gonads (1986) Obstet Gynecol Surv, 41, pp. 74-79Krasna, I.H., Lee, M.L., Smilow, P., Sciorra, L., Eierman, L., Risk of malignancy in bilateral streak gonads: The role of the Y chromosome (1992) J Pediatr Surg, 27, pp. 1376-1380Rohatgi, M., Gupta, D.K., Menon, P.S., Verma, I.C., Mathur, M., Mixed gonadal dysgenesis and dysgenetic male pseudohermaphroditism - A critical analysis (1992) Indian J Pediatr, 59, pp. 487-500Rey, R.A., Belville, C., Nhou-Fékété, C., Evaluation of gonadal function in 107 intersex patients by means of serum antimüllerian hormone measurement (1999) J Clin Endocrinol Metab, 84, pp. 627-631Stuchi-Perez, E.G., Lukas-Croisier, C., Castro, M., Evaluation of the tubular and interstitial functions of the testis in 46,XY patients with ambiguous genitalia (2000) J Pediatr Endocrinol Metab, 13, pp. 605-612Chang, H.J., Clark, R.D., Bachman, H., The phenotype of 45,X/46,XY mosaicism: An analysis of 92 prenatally diagnosed cases (1990) Am J Hum Genet, 46, pp. 156-167Rajfer, J., Walsh, P.C., Mixed gonadal dysgenesis: Dysgenetic male pseudoher-maphroditism (1981) The Intersex Child: Pediatric and Adolescent Endocrinology, pp. 103-115. , Josso N, ed. Basel, Switzerland: S. KargerBorer, J.G., Nitti, V.W., Glassberg, K.I., Mixed gonadal dysgenesis and dysgenetic male pseudohermaphroditism (1995) J Urol, 153, pp. 1267-1273Donahoe, P.K., Crawford, J.D., Hendren, W.H., Mixed gonadal dysgenesis: Pathogenesis and management (1979) J Pediatr Surg, 14, pp. 287-300Pelletier, J., Bruening, W., Kashtan, C.E., Germline mutations in the Wilms' tumor supressor gene are associated with abnormal urogenital development in Denys-Drash syndrome (1991) Cell, 67, pp. 437-1147Carré-Éusebe, D., Imbeaud, S., Harbison, M., New, M.I., Josso, N., Picard, J.Y., Variants of the anti-Müllerian hormone gene in a compound heterozygote with the persistent Müllerian duct syndrome and his family (1992) Hum Genet, 90, pp. 389-394Nistal, M., Paniagua, R., Non-neoplastic diseases of the testis (1996) Urologic Surgical Pathology, pp. 458-565. , Bostiwick DG, Eble JN, eds. St Louis, Mo: MosbyLennox, B., Ahmad, K.M., Mack, W.S., A method for determining the relative total length of the tubules in the testis (1970) J Pathol, 102, pp. 229-238Jimenez, R., Sanchez, A., Burgos, M., Dias De La Guardia, R.C., Puzzling out the genetics of mammalian sex determination (1996) Trends Genet, 12, pp. 164-166Müller, J., Skakkebaek, N.F., Quantification of germ cells and seminiferous tubules by stereological examination of testicles from 50 boys who suffered from sudden death (1983) Int J Androl, 6, pp. 143-156Cortes, D., Müller, J., Skakkebaek, N.E., Proliferation of Sertoli cells during development of the human testis assessed by stereological methods (1987) Int J Androl, 10, pp. 589-596Nistal, M., Abaurrea, M.A., Paniagua, R., Morphological and histometric study on the human Sertoli cell from birth to the onset of puberty (1982) J Anat, 14, pp. 351-363Van Niekerk, W.A., Retief, A.E., The gonads of human true hermaphrodites (1981) Hum Genet, 58, pp. 117-122Guerra Jr., G., De Mello, M.P., Assumpção, J.G., True hermaphrodites in the southeastern region of Brazil: A different cytogenetic and gonadal profile (1998) J Pediatr Endocrinol Metab, 11, pp. 519-524Quigley, C.A., De Bellis, A., Marschke, K.B., El-Awady, M.K., Wilson, E.M., French, F.S., Androgen receptor defects: Historical, clinical and molecular perspectives (1995) Endocr Rev, 16, pp. 271-32

Downregulation Of 14q32 Micrornas In Primary Human Desmoplastic Medulloblastoma

Medulloblastoma (MB) is one of the most common pediatric cancers, likely originating from abnormal development of cerebellar progenitor neurons. MicroRNA (miRNA) has been shown to play an important role in the development of the central nervous system. Microarray analysis was used to investigate miRNA expression in desmoplastic MB from patients diagnosed at a young age (1 or 2 years old). Normal fetal or newborn cerebellum was used as control. A total of 84 differentially expressed miRNAs (64 downregulated and 20 upregulated) were found. Most downregulated miRNAs (32/64) were found to belong to the cluster of miRNAs at the 14q32 locus, suggesting that this miRNA locus is regulated as a module in MB. Possible mechanisms of 14q32 miRNAs downregulation were investigated by the analysis of publicly available gene expression data sets. First, expression of estrogen-related receptor-γ (ESRRG), a reported positive transcriptional regulator of some 14q32 miRNAs, was found downregulated in desmoplastic MB. Second, expression of the parentally imprinted gene MEG3 was lower in MB in comparison to normal cerebellum, suggesting a possible epigenetic silencing of the 14q32 locus. miR-129-5p (11p11.2/7q32.1), miR-206 (6p12.2), and miR-323-3p (14q32.2), were chosen for functional studies in DAOY cells. Overexpression of miR-129-5p using mimics decreased DAOY proliferation. No effect was found with miR-206 or miR-323 mimics. © 2013 Lucon, Rocha, Craveiro, Dilloo, Cardinalli, Cavalcanti, Aguiar, Maurer-Morelli and Yunes.3 SEPEllison, D., Classifying the medulloblastoma: insights from morphology and molecular genetics (2002) Neuropathol Appl Neurobiol, 28, pp. 257-282. , doi:10.1046/j.1365-2990.2002.00419.xGulino, A., Di Marcotullio, L., Ferretti, E., De Smaele, E., Screpanti, I., Hedgehog signaling pathway in neural development and disease (2007) Psychoneuroendocrinology, 32, pp. S25-56. , doi:10.1016/j.psyneuen.2007.03.017Kool, M., Koster, J., Bunt, J., Hasselt, N.E., Lakeman, A., van Sluis, P., Integrated genomics identifies five medulloblastoma subtypes with distinct genetic profiles, pathway signatures and clinicopathological features (2008) PLoS ONE, 3, pp. e3088. , doi:10.1371/journal.pone.0003088Northcott, P.A., Korshunov, A., Witt, H., Hielscher, T., Eberhart, C.G., Mack, S., Medulloblastoma comprises four distinct molecular variants (2010) J Clin Oncol, 29, pp. 1408-1414. , doi:10.1200/JCO.2009.27.4324Gilbertson, R.J., Ellison, D.W., The origins of meduloblastoma subtypes (2008) Annu Rev Pathol, 3, pp. 341-365. , doi:10.1146/annurev.pathmechdis.3.121806.151518Schüller, U., Heine, V.M., Mao, J., Kho, A.T., Dillon, A.K., Han, Y.G., Acquisition of granule neuron precursor identity is a critical determinant of progenitor cell competence to form SHH-induced medulloblastoma (2008) Cancer Cell, 14, pp. 123-134. , doi:10.1016/j.ccr.2008.07.005Yang, Z.J., Ellis, T., Markant, S.L., Read, T.A., Kessler, J.D., Bourboulas, M., Medulloblastoma can be initiated by deletion of Patched in lineage-restricted progenitors or stem cells (2008) Cancer Cell, 14 (2), pp. 135-145. , doi:10.1016/j.ccr.2008.07.003Gibson, P., Tong, Y., Robinson, G., Thomson, M.C., Currie, D.S., Eden, C., Subtypes of medulloblastoma have distinct developmental origins (2010) Nature, 468, pp. 1095-1099. , doi:10.1038/nature09587Cohen, S.M., (2010) MiRNAs in CNS Development and Neurodegeneration: Insights from Drosophila Genetics, , http://www.springer.com/978-3-642-04297-3, Springer Available fromSood, P., Krek, A., Zavolan, M., Macino, G., Rajewsky, N., Cell-type-specific signatures of microRNAs on target mRNA expression (2006) Proc Natl Acad Sci U S A, 103 (8), pp. 2746-2751. , doi:10.1073/pnas.0511045103Guo, H., Ingolia, N.T., Weissman, J.S., Bartel, D.P., Mammalian microRNAs predominantly act to decrease target mRNA levels (2010) Nature, 466 (7308), pp. 835-841. , doi:10.1038/nature09267Ferretti, E., De Smaele, E., Po, A., Di Marcotullio, L., Tosi, E., Espínola, M.S.B., MiRNA profiling in human meduloblastoma (2009) Int J Cancer, 124, pp. 568-577. , doi:10.1002/ijc.23948Northcott, P.A., Fernandez, L.A., Hagan, J.P., Ellison, D.W., Grajkowska, W., Gillespie, Y., The miR-17/92 polycistron is upregulated in Sonic Hedgehog driven medulloblastomas and induced by N-myc in Sonic Hedgehog-treated cerebellar neural precursors (2009) Cancer Res, 69, pp. 3249-3255. , doi:10.1158/0008-5472.CAN-08-4710Cho, Y.J., Tsherniak, A., Tamayo, P., Santagata, S., Ligon, A., Greulich, H., Integrative genomic analysis of medulloblastoma identifies a molecular subgroup that drives poor clinical outcome (2011) J Clin Oncol, 29 (11), pp. 1424-1430. , doi:10 1200/JCO. 2010. 28. 5148Fernandez, L.A., Northcott, P.A., Taylor, M.D., Kenney, A.M., Normal and oncogenic roles for microRNAs in the developing brain (2009) Cell Cycle, 8 (24), pp. 4049-4054. , doi:10.4161/cc.8.24.10243Pfister, S.M., Korshunov, A., Kool, M., Hasselblatt, M., Eberhart, C., Taylor, M.D., Molecular diagnostics of CNS embryonal tumors (2010) Acta Neuropathol, 120, pp. 553-566. , doi:10.1007/s00401-010-0751-5Livak, K.J., Schmittgen, T.D., Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) method (2001) Methods, 25, pp. 402-408. , doi:10.1006/meth.2001.1262Pietsch, T., Scharmann, T., Fonatsch, C., Schmidt, D., Ockler, R., Freihoff, D., Characterization of five new cell lines derived from human primitive neuroectodermal tumors of the central nervous system (1994) Cancer Res, 54, pp. 3278-3287Breitling, R., Armengaud, P., Amtmann, A., Herzyk, P., Rank products: a simple, yet powerful, new method to detect differentially regulated genes in replicated microarray experiments (2004) FEBS Lett, 573, pp. 83-92. , doi:10.1016/j.febslet.2004.07.055Gautier, L., Cope, L., Bolstad, B.M., Irizarry, R.A., Affy - analysis of Affymetrix GeneChip data at the probe level (2004) Bioinformatics, 20, pp. 307-315. , doi:10.1093/bioinformatics/btg405Gentleman, R.C., Carey, V.J., Bates, D.M., Bolstad, B., Dettling, M., Dudoit, S., Bioconductor: open software development for computational biology and bioinformatics (2004) Genome Biol, 5, pp. R80Zhou, J., Tian, Y., Li, J., Lu, B., Sun, M., Zou, Y., miR-206 is down-regulated in breast cancer and inhibits cell proliferation through the up-regulation of cyclinD2 (2013) Biochem Biophys Res Commun, 433 (2), pp. 207-212. , doi:10.1016/j.bbrc.2013.02.084Birks, D.K., Barton, V.N., Donson, A.M., Handler, M.H., Vibhakar, R., Foreman, N.K., Survey of MicroRNA expression in pediatric brain tumors (2011) Pediatr Blood Cancer, 56 (2), pp. 211-216. , doi:10.1002/pbc.22723Huang, Y.W., Liu, J.C., Deatherage, D.E., Luo, J., Mutch, D.G., Goodfellow, P.J., Epigenetic repression of microRNA-129-2 leads to upregulation of SOX4 oncogene in endometrial cancer (2009) Cancer Res, 69 (23), pp. 9038-9046. , doi:10.1158/0008-5472.CAN-09-1499Shen, R., Pan, S., Qi, S., Lin, X., Cheng, S., Epigenetic repression of microRNA-129-2 leads to upregulation of SOX4 in gastric cancer (2010) Biochem Biophys Res Commun, 394 (4), pp. 1047-1052. , doi:10.1016/j.bbrc.2010.03.121Neben, K., Korshunov, A., Benner, A., Wrobel, G., Hahn, M., Kokocinski, F., Microarray-based screening for molecular markers in medulloblastoma revealed STK15 as independent predictor for survival (2004) Cancer Res, 64 (9), pp. 3103-3111. , doi:10.1158/0008-5472.CAN-03-3968de Bont, J.M., Kros, J.M., Passier, M.M., Reddingius, R.E., Sillevis Smitt, P.A., Luider, T.M., Differential expression and prognostic significance of SOX genes in pediatric medulloblastoma and ependymoma identified by microarray analysis (2008) Neuro Oncol, 10 (5), pp. 648-660. , doi:10.1215/15228517-2008-032Garzia, L., Andolfo, I., Cusanelli, E., Marino, N., Petrosino, G., De Martino, D., MicroRNA-199b-5p impairs cancer stem cells through negative regulation of HES1 in medulloblastoma (2009) PLoS ONE, 4, p. 4998. , doi:10.1371/journal.pone.0004998Gokhale, A., Kunder, R., Goel, A., Sarin, R., Moiyadi, A., Shenoy, A., Distinctive microRNA signature of medulloblastomas associated with the WNT signaling pathway (2010) J Cancer Res Ther, 6, pp. 521-529. , doi:10.4103/0973-1482.77072de Antonellis, P., Medaglia, C., Cusanelli, E., Andolfo, I., Liguori, L., De Vita, G., MiR-34a targeting of notch ligand delta-like 1 impairs CD15+/CD133+ tumor-propagating cells and supports neural differentiation in medulloblastoma (2011) PLoS ONE, 6 (9), pp. e24584. , doi:10.1371/journal.pone.0024584Grunder, E., D'Ambrosio, R., Fiaschetti, G., Abela, L., Arcaro, A., Zuzak, T., MicroRNA-21 suppression impedes medulloblastoma cell migration (2011) Eur J Cancer, 47, pp. 2479-2490. , doi:10.1016/j.ejca.2011.06.041Uziel, T., Karginov, F.V., Xie, S., Parker, J.S., Wang, Y.D., Gajjar, A., The miR-17 92 cluster collaborates with the Sonic Hedgehog pathway in medulloblastoma (2009) Proc Natl Acad Sci USA, 106, pp. 2812-2817. , doi:10.1073/pnas.0809579106Chédotal, A., Kerjan, G., Moreau-Fauvarque, C., The brain within the tumor: new roles for axon guidance molecules in cancers (2005) Cell Death Differ, 12 (8), pp. 1044-1056. , doi:10.1038/sj.cdd.4401707Aref, D., Moffatt, C.J., Agnihotri, S., Ramaswamy, V., Dubuc, A.M., Northcott, P.A., Canonical TGF-b pathway activity is a predictor of SHH-driven medulloblastoma survival and delineates putative precursors in cerebellar development (2013) Brain Pathol, 23, pp. 178-191. , doi:10.1111/j.1750-3639.2012.00631.xLuo, X., Liu, J., Cheng, S.Y., The role of microRNAs during the genesis of medulloblastoma induced by the hedgehog pathway (2011) Biomed Res, 25 (1), pp. 42-48. , doi:10. 1016/S1674-8301(11)Northcott, P.A., Shih, D.J., Peacock, J., Garzia, L., Morrissy, A.S., Zichner, T., Subgroup-specific structural variation across 1,000 medulloblastoma genomes (2012) Nature, 488 (7409), pp. 49-56. , doi:10.1038/nature11327Song, G., Wang, L., miR-433 and miR-127 arise from independent overlapping primary transcripts encoded by the miR-433-127 locus (2008) PLoS ONE, 3, pp. e3574. , doi:10.1371/journal.pone.0003574Remke, M., Hielscher, T., Korshunov, A., Northcott, P.A., Bender, S., Kool, M., FSTL5 is a marker of poor prognosis in non-WNT/non-SHH medulloblastoma (2011) J Clin Oncol, 29 (29), pp. 3852-3861. , doi:10.1200/JCO.2011.36.2798Edgar, R., Domrachev, M., Lash, A.E., Gene expression omnibus: NCBI gene expression and hybridization array data repository (2002) Nucleic Acids Res, 30, pp. 207-210. , doi:10.1093/nar/30.1.207Yu, S., Wang, X., Ng, C.F., Chen, S., Chan, F.L., ERRgamma suppresses cell proliferation and tumor growth of androgen-sensitive and androgen-insensitive prostate cancer cells and its implication as a therapeutic target for prostate cancer (2007) Cancer Res, 67 (10), pp. 4904-4914. , doi:10.1158/0008-5472.CAN-06-3855Mancuso, M., Leonardi, S., Giardullo, P., Pasquali, E., Borra, F., Stefano, I.D., The estrogen receptor beta agonist diarylpropionitrile (DPN) inhibits medulloblastoma development via anti-proliferative and pro-apototic pathways (2011) Cancer Lett, 308 (2), pp. 197-202. , doi:10.1016/j.canlet.2011.05.004da Rocha, S.T., Edwards, C.A., Ito, M., Ogata, T., Ferguson-Smith, A.C., Genomic imprinting at the mammalian Dlk1-Dio3 domain (2008) Trends Genet, 24, pp. 306-316. , doi:10.1016/j.tig.2008.03.011Lin, S.P., Youngson, N., Takada, S., Seitz, H., Reik, W., Paulsen, M., Asymmetric regulation of imprinting on the maternal and paternal chromosomes at the Dlk1-Gtl2 imprinted cluster on mouse chromosome 12 (2003) Nat Genet, 35, pp. 97-102. , doi:10.1038/ng1233Takada, S., Paulsen, M., Tevendale, M., Tsai, C.E., Kelsey, G., Cattanach, B.M., Epigenetic analysis of the Dlk1-Gtl2 imprinted domain on mouse chromosome implications for imprinting control from comparison with Igf2-H19 (2002) Hum Mol Genet, 12, pp. 77-86. , doi:10.1093/hmg/11.1.77Kagami, M., O'Sullivan, M.J., Green, A.J., Watabe, Y., Arisaka, O., Masawa, N., The IG-DMR and the MEG3-DMR at human chromosome 14q32 2: hierarchical interaction and distinct functional properties as imprinting control centers (2010) PLoS Genet, 6, pp. e1000992. , doi:10.1371/journal.pgen.1000992Diede, S.J., Guenthoer, J., Geng, L.N., Mahoney, S.E., Marotta, M., Olson, J.M., DNA methylation of developmental genes in pediatric medulloblastomas identified by denaturation analysis of methylation differences (2010) Proc Natl Acad Sci U S A, 107 (1), pp. 234-239. , doi:10.1073/pnas.0907606106Thayanithy, V., Park, C., Sarver, A.L., Kartha, R.V., Korpela, D.M., Graef, A.J., Combinatorial treatment of DNA and chromatin-modifying drugs cause cell death in human and canine osteosarcoma cell lines (2012) PLoS ONE, 7 (9), pp. e43720. , doi:10.1371/journal.pone.0043720Pierson, J., Hostager, B., Fan, R., Vibhakar, R., Regulation of cyclin dependent kinase 6 by microRNA 124 in medulloblastoma (2008) J Neurooncol, 90, pp. 1-7. , doi:10.1007/s11060-008-9624-3Li, K.K., Pang, J.C., Ching, A.K., Wong, C.K., Kong, X., Wang, Y., miR-124 is frequently down-regulated in meduloblastoma and is a negative regulator of SLC16A1 (2009) Hum Pathol, 40 (9), pp. 1234-1243. , doi:10.1016/j.humpath.2009.02.003Ferretti, E., De Smaele, E., Miele, E., Laneve, P., Pó, A., Pelloni, M., Concerted miRNA control of Hedgehog signalling in cerebellar neuronal progenitor and tumour cells (2008) EMBO J, 27, pp. 2616-2627. , doi:10.1038/emboj.2008.172Venkataraman, S., Alimova, I., Fan, R., Harris, P., Foreman, N., Vibhakar, R., MicroRNA 128a increases intracellular ROS level by targeting Bmi-1 and inhibits medulloblastoma cancer cell growth by promoting senescence (2010) PLoS ONE, 5 (6), pp. e10748. , doi:10.1371/journal.pone.0010748Genovesi, L.A., Carter, K.W., Gottardo, N.G., Giles, K.M., Dallas, P.B., Integrated analysis of miRNA and mRNA expression in childhood medulloblastoma compared with neural stem cells (2011) PLoS ONE, 6 (9), pp. e23935. , doi:10.1371/journal.pone.0023935Weeraratne, S.D., Amani, V., Neiss, A., Teider, N., Scott, D.K., Pomeroy, S.L., miR-34a confers chemosensitivity through modulation of MAGE-A and p53 in medulloblastoma (2011) Neuro Oncol, 13 (2), pp. 165-175. , doi:10.1093/neuonc/noq179Lv, S.Q., Kim, Y.H., Giulio, F., Shalaby, T., Nobusawa, S., Yang, H., Genetic alterations in MicroRNAs in medulloblastomas (2012) Brain Pathol, 22, pp. 230-239. , doi:10.1111/j.1750-3639.2011.00523.xWang, X.M., Zhang, S.F., Cheng, Z.Q., Peng, Q.Z., Hu, J.T., Gao, L.K., MicroRNA383 regulates expression of PRDX3 in human medulloblastomas (2012) Zhonghua Bing Li Xue Za Zhi, 41 (8), pp. 547-552. , doi:10.3760/cma.j.issn.0529-5807.2012.08.009Li, K.K.W., Pang, J.C.S., Lau, K.M., Zhou, L., Mao, Y., Wang, Y., MiR-383 is downregulated in medulloblastoma and targets peroxiredoxin 3 (PRDX3) (2013) Brain Pathol, 23 (4), pp. 413-425. , doi:10.1111/bpa.12014Weeraratne, S.D., Amani, V., Teider, N., Pierre-Francois, J., Winter, D., Kye, M.J., Pleiotropic effects of miR-183 96 182 converge to regulate cell survival, proliferation and migration in meduloblastoma (2012) Acta Neuropathol, 123, pp. 539-552. , doi:10.1007/s00401-012-0969-5Venkataraman, S., Birks, D.K., Balakrishnan, I., Alimova, I., Harris, P.S., Patel, P.R., MicroRNA 218 acts as a tumor suppressor by targeting multiple cancer phenotype-associated genes in medulloblastoma (2013) J Biol Chem, 228 (3), pp. 1918-1928. , doi:10.1074/jbc.M112.396762Wei, L., Yan-hua, G., Teng-fei, C., Xiao-zhong, P., Jian-gang, Y., Zhen-yu, M., Identification of differentially expressed microRNAs by microarray: a possible role for microRNAs gene in medulloblastomas (2009) Chin Med J, 122 (20), pp. 2405-241

Granulocytic Sarcoma Of The Larynx Preceding Chronic Myeloid Leukemia

The authors report one case of granulocytic sarcoma infiltrating the larynx and cervical lymph nodes in a 50-year-old smoking patient. At the time of diagnosis there was no clinical and laboratory evidence of acute myeloid leukemia or chronic myeloproliferative disease. Four months after diagnosis, bone marrow morphology was consistent with chronic myeloid leukemia, accelerated phase. Cytogenetic abnormalities (Ph 1 chromosome, t (1; 12)(p36; p13), and trisomy of chromosome 20) were also found in hemopoetic cells. Granulocytic sarcoma preceding installation of chronic myeloid leukemia, as described here, seems to be a rare clinical event.18991084108

Renal Medullary Carcinoma: Report Of Seven Cases From Brazil

We report seven cases of renal medullary carcinoma collected from several institutions in Brazil. In spite of a relatively high incidence of sickle cell trait in Brazil, this is a rare tumor. All patients were males between the ages of 8 and 69 years (mean 22 years). From the collected information, the most frequent presenting symptoms were gross hematuria and flank or abdominal pain. The duration of symptoms ranged from 1 week to 5 months. Most of the tumors were poorly circumscribed arising centrally in the renal medulla. Size ranged from 4 to 12 cm (mean 7 cm) and hemorrhage and necrosis were common findings. All seven cases described showed sickled red blood cells in the tissue and six patients were confirmed to have sickle cell trait. All cases disclosed the characteristic reticular pattern consisting of tumor cell aggregates forming spaces of varied size, reminiscent of yolk sac testicular tumors of reticular type. Other findings included microcystic, tubular, trabecular, solid and adenoid-cystic patterns, rhabdoid-like cells and stromal desmoplasia. A peculiar feature was suppurative necrosis typically resembling microabscesses within epithelial aggregates. The medullary carcinoma of the 69-year-old patient was associated with a conventional clear cell carcinoma. To our knowledge, this association has not been previously reported and the patient is the oldest in the literature. The survival after diagnosis or admission ranged from 4 days to 9 months. The 8-year-old African-Brazilian patient with a circumscribed mass is alive and free of recurrence 8 years after diagnosis. This case raises the question whether a periodic search for renal medullary carcinoma in young patients who have known abnormalities of the hemoglobin gene and hematuria could result in an early diagnosis and a better survival. © 2007 USCAP, Inc All rights reserved.209914920Davis Jr, C.J., Mostofi, F.K., Sesterhenn, I.A., Renal medullary carcinoma. The seventh sickle cell nephropathy (1995) Am J Surg Pathol, 19, pp. 1-11Leitao, V.A., Silva Jr, W., Ferreira, U., Renal medullary carcinoma. Case report and review of the literature (2006) Urol Int, 77, pp. 184-186Hsu, S.M., Raine, L., Fanger, H., Use of avidin-biotin-peroxidase complex (ABC) in immunoperoxidase techniques. A comparison between ABC labeled antibody (PAP) procedures (1981) J Histochem Cytochem, 29, pp. 557-589Berman, L.B., Sickle cell nephropathy (1974) JAMA, 228, p. 1279Beutler, E., Disorders of hemoglobin structure: Sickle cell anemia and related abnormalities (2006) Williams Hematology, pp. 667-700. , Lichtman MA, Beutler E, Kipps TJ, Seligsohn U, Kaushansky K, Prchal JT eds, 7th edn. McGraw-Hill: New-YorkKhan, A., Thomas, N., Costello, B., Renal medullary carcinoma: Sonographic, computed tomography, magnetic resonance and angiographic findings (2000) Eur J Radiol, 35, pp. 1-7Simpson, L., He, X., Pins, M., Renal medullary carcinoma and ABL gene amplification (2005) J Urol, 173, pp. 1883-1888Chapadeiro E, Maciel R, Jamra M, et al. Linfonodos, baço, medula óssea e sangue. Tumores do Sistema Hemolinfático. Timo. In: Lopes ER, Chapadeiro E, Raso P, Tafuri WL (eds). Bogliolo Patologia, 4th edn. Guanabara Koogan: Rio de Janeiro, 1987, pp 651-652Davis Jr, C.J., Renal medullary carcinoma (2004) World Health Organization Classification of Tumours, Pathology and Genetics of Tumors of the Urinary System and Male Genital Organs, pp. 162-192. , Eble JN, Sauter G, Epstein JI, et al eds, IARC Press: LyonSwartz, M.A., Karth, J., Schneider, D.T., Renal medullary carcinoma: Clinical, pathologic, immunohistochemical, and genetic analysis with pathogenetic implications (2002) Ped Urol, 60, pp. 1083-1089Rodriguez-Jurado, R., Gonzalez-Crussi, F., Renal medullary carcinoma (1996) J Urol Pathol, 4, pp. 191-203Srigley, J.R., Eble, J.N., Collecting duct carcinoma of kidney (1998) Semin Diagn Pathol, 15, pp. 54-67Kennedy, S.M., Merino, M.J., Linehan, W.M., Collecting duct carcinoma of the kidney (1990) Hum Pathol, 21, pp. 449-456Amin, M.B., Varma, M.D., Tickoo, S.K., Collecting duct carcinoma of the kidney (1997) Adv Anat Pathol, 4, pp. 85-94Yang, X.J., Sugimura, J., Tretiakova, M.S., Gene expression profiling of renal medullary carcinoma: Potential clinical relevance (2004) Cancer, 100, pp. 976-985Figenshau, R.S., Basler, J.W., Ritter, J.H., Renal medullary carcinoma (1998) J Urol, 159, pp. 711-713Pisani, P., Bray, F., Parkin, D.M., Estimates of the worldwide prevalence of cancer for 25 sites in the adult population (2002) Int J Cancer, 97, pp. 72-8

Langerhans cell histiocytosis and its relationship with epstein-barr virus

[No abstract available]37111508150