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105 results on '"Robinson, Giles W"'

2. Concurrent ependymal and ganglionic differentiation in a subset of supratentorial neuroepithelial tumors with EWSR1-PLAGL1 rearrangement

Author

Lee, Julieann C., Koo, Selene C., Furtado, Larissa V., Breuer, Alex, Eldomery, Mohammad K., Bag, Asim K., Stow, Pat, Rose, Gary, Larkin, Trisha, Sances, Rick, Kleinschmidt-DeMasters, Bette K., Bodmer, Jenna L., Willard, Nicholas, Gokden, Murat, Dahiya, Sonika, Roberts, Kaleigh, Bertrand, Kelsey C., Moreira, Daniel C., Robinson, Giles W., Mo, Jun Qin, Ellison, David W., and Orr, Brent A.

Published
2024
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3. Molecular classification and outcome of children with rare CNS embryonal tumors: results from St. Jude Children’s Research Hospital including the multi-center SJYC07 and SJMB03 clinical trials

Author

Liu, Anthony P. Y., Dhanda, Sandeep K., Lin, Tong, Sioson, Edgar, Vasilyeva, Aksana, Gudenas, Brian, Tatevossian, Ruth G., Jia, Sujuan, Neale, Geoffrey, Bowers, Daniel C., Hassall, Tim, Partap, Sonia, Crawford, John R., Chintagumpala, Murali, Bouffet, Eric, McCowage, Geoff, Broniscer, Alberto, Qaddoumi, Ibrahim, Armstrong, Greg, Wright, Karen D., Upadhyaya, Santhosh A., Vinitsky, Anna, Tinkle, Christopher L., Lucas, John, Chiang, Jason, Indelicato, Daniel J., Sanders, Robert, Klimo, Jr., Paul, Boop, Frederick A., Merchant, Thomas E., Ellison, David W., Northcott, Paul A., Orr, Brent A., Zhou, Xin, Onar-Thomas, Arzu, Gajjar, Amar, and Robinson, Giles W.

Published
2022
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4. Unified rhombic lip origins of group 3 and group 4 medulloblastoma

Author

Smith, Kyle S., Bihannic, Laure, Gudenas, Brian L., Haldipur, Parthiv, Tao, Ran, Gao, Qingsong, Li, Yiran, Aldinger, Kimberly A., Iskusnykh, Igor Y., Chizhikov, Victor V., Scoggins, Matthew, Zhang, Silu, Edwards, Angela, Deng, Mei, Glass, Ian A., Overman, Lynne M., Millman, Jake, Sjoboen, Alexandria H., Hadley, Jennifer, Golser, Joseph, Mankad, Kshitij, Sheppard, Heather, Onar-Thomas, Arzu, Gajjar, Amar, Robinson, Giles W., Hovestadt, Volker, Orr, Brent A., Patay, Zoltán, Millen, Kathleen J., and Northcott, Paul A.

Published
2022
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7. Outcomes for children with recurrent/refractory atypical teratoid rhabdoid tumor: A single‐institution study with molecular correlation.

Author

Carey, Steven S., Huang, Jie, Myers, Jason R., Mostafavi, Roya, Orr, Brent A., Dhanda, Sandeep Kumar, Michalik, Layna H., Tatevossian, Ruth G., Klimo, Paul, Boop, Frederick, Lu, Congyu, Sioson, Edgar, Zhou, Xin, Nichols, Kim E., Merchant, Thomas E., Ellison, David W., Robinson, Giles W., Onar‐Thomas, Arzu, Gajjar, Amar, and Upadhyaya, Santhosh A.

Published
2024
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8. Intensive Interdisciplinary Rehabilitation in the Pediatric Hematology/Oncology Setting: Feasibility and Perceived Benefit of the Acute Neurological Injury Service.

Author

Raches, Darcy, Gajjar, Amar, Robinson, Giles W., Ashford, Jason M., Bryndziar, Martina, Huggins, April, Lockett, Sherry, Harris, Allison, Taylor, Hannah, Bursi, Ellen, and Conklin, Heather M.

Subjects
REHABILITATION for brain injury patients, ACUTE diseases, HOSPITAL care, PILOT projects, ONCOLOGY, TERTIARY care, GOAL (Psychology), MENTORING, HEMATOLOGY, CONVALESCENCE, COMMUNICATION, TRANSITIONAL programs (Education), CHILDREN
Abstract

Simple Summary: Interdisciplinary rehabilitation more effectively promotes recovery from acquired brain injury than a single discipline approach. Little is known about the desirability and benefit of this approach for pediatric cancer patients. We show that an interdisciplinary approach within a specialized pediatric hematology/oncology hospital is manageable for caregivers of children with new cancer diagnoses. Parents found this approach helpful, including coordinated care planning, setting an interdisciplinary goal, parent brain injury education, cognitive assessment reports, and weekly cognitive intervention sessions. Parents wanted to have a peer mentor while managing new cancer diagnoses and later serve as a mentor for a newly diagnosed family. Other benefits of this approach should be explored. (1) Background: Intensive interdisciplinary rehabilitation services more effectively promote recovery from acquired brain injury than a single discipline approach. However, research literature is lacking regarding the perceived feasibility and utility of an interdisciplinary approach across disciplines for patients within a tertiary care pediatric hematology/oncology setting. (2) Methods: The Acute Neurological Injury (ANI) service applied an acquired brain injury/inpatient rehabilitation interdisciplinary approach to a pediatric hematology/oncology population, with a focus on interdisciplinary communication, shared goal setting, and coordinated transition planning. Caregivers whose children received coordinated ANI program care were interviewed regarding the perceived feasibility and utility of ANI program components. (3) Results: An interdisciplinary approach to a pediatric hematology/oncology population is feasible for caregivers and for providers of rehabilitation and psychosocial services within a tertiary care cancer hospital setting. Parents perceived benefits from aspects of this approach including coordinated interdisciplinary care planning, the implementation of an interdisciplinary goal, parent brain injury education, neuropsychological assessment reports, and weekly cognitive intervention sessions. Parents were interested in both having a peer mentor while managing new cancer diagnoses and later serving in a mentor role for a newly diagnosed family. (4) Conclusions: An interdisciplinary acquired brain injury approach to a pediatric hematology/oncology population is feasible with perceived benefits to families managing new cancer diagnoses. [ABSTRACT FROM AUTHOR]

Published
2024
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9. Clinical and molecular heterogeneity of pineal parenchymal tumors: a consensus study

Author

Liu, Anthony P. Y., Li, Bryan K., Pfaff, Elke, Gudenas, Brian, Vasiljevic, Alexandre, Orr, Brent A., Dufour, Christelle, Snuderl, Matija, Karajannis, Matthias A., Rosenblum, Marc K., Hwang, Eugene I., Ng, Ho-Keung, Hansford, Jordan R., Szathmari, Alexandru, Faure-Conter, Cécile, Merchant, Thomas E., Levine, Max, Bouvier, Nancy, von Hoff, Katja, Mynarek, Martin, Rutkowski, Stefan, Sahm, Felix, Kool, Marcel, Hawkins, Cynthia, Onar-Thomas, Arzu, Robinson, Giles W., Gajjar, Amar, Pfister, Stefan M., Bouffet, Eric, Northcott, Paul A., Jones, David T. W., and Huang, Annie

Published
2021
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12. Patient-derived models recapitulate heterogeneity of molecular signatures and drug response in pediatric high-grade glioma

Author

He, Chen, Xu, Ke, Zhu, Xiaoyan, Dunphy, Paige S., Gudenas, Brian, Lin, Wenwei, Twarog, Nathaniel, Hover, Laura D., Kwon, Chang-Hyuk, Kasper, Lawryn H., Zhang, Junyuan, Li, Xiaoyu, Dalton, James, Jonchere, Barbara, Mercer, Kimberly S., Currier, Duane G., Caufield, William, Wang, Yingzhe, Xie, Jia, Broniscer, Alberto, Wetmore, Cynthia, Upadhyaya, Santhosh A., Qaddoumi, Ibrahim, Klimo, Paul, Boop, Frederick, Gajjar, Amar, Zhang, Jinghui, Orr, Brent A., Robinson, Giles W., Monje, Michelle, Freeman III, Burgess B., Roussel, Martine F., Northcott, Paul A., Chen, Taosheng, Rankovic, Zoran, Wu, Gang, Chiang, Jason, Tinkle, Christopher L., Shelat, Anang A., and Baker, Suzanne J.

Published
2021
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13. Germline Elongator mutations in Sonic Hedgehog medulloblastoma

Author

Waszak, Sebastian M., Robinson, Giles W, Gudenas, Brian L., Smith, Kyle S., Forget, Antoine, Kojic, Marija, and Garcia-Lopez, Jesus

Subjects
Gene mutations -- Physiological aspects -- Genetic aspects, Medulloblastoma -- Development and progression -- Genetic aspects -- Physiological aspects, Translation elongation factors -- Genetic aspects -- Physiological aspects, Tumors in children -- Development and progression -- Genetic aspects -- Physiological aspects, Environmental issues, Science and technology, Zoology and wildlife conservation
Abstract

Cancer genomics has revealed many genes and core molecular processes that contribute to human malignancies, but the genetic and molecular bases of many rare cancers remains unclear. Genetic predisposition accounts for 5 to 10% of cancer diagnoses in children.sup.1,2, and genetic events that cooperate with known somatic driver events are poorly understood. Pathogenic germline variants in established cancer predisposition genes have been recently identified in 5% of patients with the malignant brain tumour medulloblastoma.sup.3. Here, by analysing all protein-coding genes, we identify and replicate rare germline loss-of-function variants across ELP1 in 14% of paediatric patients with the medulloblastoma subgroup Sonic Hedgehog (MB.sub.SHH).sub..ELP1 was the most common medulloblastoma predisposition gene and increased the prevalence of genetic predisposition to 40% among paediatric patients with MB.sub.SHH. Parent-offspring and pedigree analyses identified two families with a history of paediatric medulloblastoma. ELP1-associated medulloblastomas were restricted to the molecular SHH[alpha] subtype.sup.4 and characterized by universal biallelic inactivation of ELP1 owing to somatic loss of chromosome arm 9q. Most ELP1-associated medulloblastomas also exhibited somatic alterations in PTCH1, which suggests that germline ELP1 loss-of-function variants predispose individuals to tumour development in combination with constitutive activation of SHH signalling. ELP1 is the largest subunit of the evolutionarily conserved Elongator complex, which catalyses translational elongation through tRNA modifications at the wobble (U.sub.34) position.sup.5,6. Tumours from patients with ELP1-associated MB.sub.SHH were characterized by a destabilized Elongator complex, loss of Elongator-dependent tRNA modifications, codon-dependent translational reprogramming, and induction of the unfolded protein response, consistent with loss of protein homeostasis due to Elongator deficiency in model systems.sup.7-9. Thus, genetic predisposition to proteome instability may be a determinant in the pathogenesis of paediatric brain cancers. These results support investigation of the role of protein homeostasis in other cancer types and potential for therapeutic interference. Germline mutations in the Elongator complex gene ELP1 predispose individuals to the development of childhood medulloblastoma., Author(s): Sebastian M. Waszak [sup.1] , Giles W Robinson [sup.2] , Brian L. Gudenas [sup.3] , Kyle S. Smith [sup.3] , Antoine Forget [sup.4] , Marija Kojic [sup.5] , Jesus [...]

Published
2020
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15. Risk-adapted therapy and biological heterogeneity in pineoblastoma: integrated clinico-pathological analysis from the prospective, multi-center SJMB03 and SJYC07 trials

Author

Liu, Anthony P. Y., Gudenas, Brian, Lin, Tong, Orr, Brent A., Klimo, Jr., Paul, Kumar, Rahul, Bouffet, Eric, Gururangan, Sridharan, Crawford, John R., Kellie, Stewart J., Chintagumpala, Murali, Fisher, Michael J., Bowers, Daniel C., Hassall, Tim, Indelicato, Daniel J., Onar-Thomas, Arzu, Ellison, David W., Boop, Frederick A., Merchant, Thomas E., Robinson, Giles W., Northcott, Paul A., and Gajjar, Amar

Published
2020
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18. Second-generation molecular subgrouping of medulloblastoma: an international meta-analysis of Group 3 and Group 4 subtypes

Author

Sharma, Tanvi, Schwalbe, Edward C., Williamson, Daniel, Sill, Martin, Hovestadt, Volker, Mynarek, Martin, Rutkowski, Stefan, Robinson, Giles W., Gajjar, Amar, Cavalli, Florence, Ramaswamy, Vijay, Taylor, Michael D., Lindsey, Janet C., Hill, Rebecca M., Jäger, Natalie, Korshunov, Andrey, Hicks, Debbie, Bailey, Simon, Kool, Marcel, Chavez, Lukas, Northcott, Paul A., Pfister, Stefan M., and Clifford, Steven C.

Published
2019
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20. DNA methylation-based classification of central nervous system tumours

Author

Capper, David, Jones, David T. W., Sill, Martin, Hovestadt, Volker, Schrimpf, Daniel, Sturm, Dominik, Koelsche, Christian, Sahm, Felix, Chavez, Lukas, Reuss, David E., Kratz, Annekathrin, Wefers, Annika K., Huang, Kristin, Pajtler, Kristian W., Schweizer, Leonille, Stichel, Damian, Olar, Adriana, Engel, Nils W., Lindenberg, Kerstin, Harter, Patrick N., Braczynski, Anne K., Plate, Karl H., Dohmen, Hildegard, Garvalov, Boyan K., Coras, Roland, Hölsken, Annett, Hewer, Ekkehard, Bewerunge-Hudler, Melanie, Schick, Matthias, Fischer, Roger, Beschorner, Rudi, Schittenhelm, Jens, Staszewski, Ori, Wani, Khalida, Varlet, Pascale, Pages, Melanie, Temming, Petra, Lohmann, Dietmar, Selt, Florian, Witt, Hendrik, Milde, Till, Witt, Olaf, Aronica, Eleonora, Giangaspero, Felice, Rushing, Elisabeth, Scheurlen, Wolfram, Geisenberger, Christoph, Rodriguez, Fausto J., Becker, Albert, Preusser, Matthias, Haberler, Christine, Bjerkvig, Rolf, Cryan, Jane, Farrell, Michael, Deckert, Martina, Hench, Jürgen, Frank, Stephan, Serrano, Jonathan, Kannan, Kasthuri, Tsirigos, Aristotelis, Brück, Wolfgang, Hofer, Silvia, Brehmer, Stefanie, Seiz-Rosenhagen, Marcel, Hänggi, Daniel, Hans, Volkmar, Rozsnoki, Stephanie, Hansford, Jordan R., Kohlhof, Patricia, Kristensen, Bjarne W., Lechner, Matt, Lopes, Beatriz, Mawrin, Christian, Ketter, Ralf, Kulozik, Andreas, Khatib, Ziad, Heppner, Frank, Koch, Arend, Jouvet, Anne, Keohane, Catherine, Mühleisen, Helmut, Mueller, Wolf, Pohl, Ute, Prinz, Marco, Benner, Axel, Zapatka, Marc, Gottardo, Nicholas G., Driever, Pablo Hernáiz, Kramm, Christof M., Müller, Hermann L., Rutkowski, Stefan, von Hoff, Katja, Frühwald, Michael C., Gnekow, Astrid, Fleischhack, Gudrun, Tippelt, Stephan, Calaminus, Gabriele, Monoranu, Camelia-Maria, Perry, Arie, Jones, Chris, Jacques, Thomas S., Radlwimmer, Bernhard, Gessi, Marco, Pietsch, Torsten, Schramm, Johannes, Schackert, Gabriele, Westphal, Manfred, Reifenberger, Guido, Wesseling, Pieter, Weller, Michael, Collins, Vincent Peter, Blümcke, Ingmar, Bendszus, Martin, Debus, Jürgen, Huang, Annie, Jabado, Nada, Northcott, Paul A., Paulus, Werner, Gajjar, Amar, Robinson, Giles W., Taylor, Michael D., Jaunmuktane, Zane, Ryzhova, Marina, Platten, Michael, Unterberg, Andreas, Wick, Wolfgang, Karajannis, Matthias A., Mittelbronn, Michel, Acker, Till, Hartmann, Christian, Aldape, Kenneth, Schüller, Ulrich, Buslei, Rolf, Lichter, Peter, Kool, Marcel, Herold-Mende, Christel, Ellison, David W., Hasselblatt, Martin, Snuderl, Matija, Brandner, Sebastian, Korshunov, Andrey, von Deimling, Andreas, and Pfister, Stefan M.

Published
2018
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22. The whole-genome landscape of medulloblastoma subtypes

Author

Northcott, Paul A., Buchhalter, Ivo, Morrissy, A. Sorana, Hovestadt, Volker, Weischenfeldt, Joachim, Ehrenberger, Tobias, Grbner, Susanne, Segura-Wang, Maia, Zichner, Thomas, Rudneva, Vasilisa A., Warnatz, Hans-Jrg, Sidiropoulos, Nikos, Phillips, Aaron H., Schumacher, Steven, Kleinheinz, Kortine, Waszak, Sebastian M., Erkek, Serap, Jones, David T. W., Worst, Barbara C., Kool, Marcel, Zapatka, Marc, Jger, Natalie, Chavez, Lukas, Hutter, Barbara, Bieg, Matthias, Paramasivam, Nagarajan, Heinold, Michael, Gu, Zuguang, Ishaque, Naveed, Jger-Schmidt, Christina, Imbusch, Charles D., Jugold, Alke, Hbschmann, Daniel, Risch, Thomas, Amstislavskiy, Vyacheslav, Gonzalez, Francisco German Rodriguez, Weber, Ursula D., Wolf, Stephan, Robinson, Giles W., Zhou, Xin, Wu, Gang, Finkelstein, David, Liu, Yanling, Cavalli, Florence M. G., Luu, Betty, Ramaswamy, Vijay, Wu, Xiaochong, Koster, Jan, Ryzhova, Marina, Cho, Yoon-Jae, Pomeroy, Scott L., Herold-Mende, Christel, Schuhmann, Martin, Ebinger, Martin, Liau, Linda M., Mora, Jaume, McLendon, Roger E., Jabado, Nada, Kumabe, Toshihiro, Chuah, Eric, Ma, Yussanne, Moore, Richard A., Mungall, Andrew J., Mungall, Karen L., Thiessen, Nina, Tse, Kane, Wong, Tina, Jones, Steven J. M., Witt, Olaf, Milde, Till, Von Deimling, Andreas, Capper, David, Korshunov, Andrey, Yaspo, Marie-Laure, Kriwacki, Richard, Gajjar, Amar, Zhang, Jinghui, Beroukhim, Rameen, Fraenkel, Ernest, Korbel, Jan O., Brors, Benedikt, Schlesner, Matthias, Eils, Roland, Marra, Marco A., Pfister, Stefan M., Taylor, Michael D., and Lichter, Peter

Subjects
DNA sequencing -- Research, Medical research, Genomes -- Research, Medulloblastoma -- Care and treatment -- Genetic aspects -- Varieties, Environmental issues, Science and technology, Zoology and wildlife conservation
Abstract

Current therapies for medulloblastoma, a highly malignant childhood brain tumour, impose debilitating effects on the developing child, and highlight the need for molecularly targeted treatments with reduced toxicity. Previous studies have been unable to identify the full spectrum of driver genes and molecular processes that operate in medulloblastoma subgroups. Here we analyse the somatic landscape across 491 sequenced medulloblastoma samples and the molecular heterogeneity among 1,256 epigenetically analysed cases, and identify subgroup-specific driver alterations that include previously undiscovered actionable targets. Driver mutations were confidently assigned to most patients belonging to Group 3 and Group 4 medulloblastoma subgroups, greatly enhancing previous knowledge. New molecular subtypes were differentially enriched for specific driver events, including hotspot in-frame insertions that target KBTBD4 and enhancer hijacking events that activate PRDM6. Thus, the application of integrative genomics to an extensive cohort of clinical samples derived from a single childhood cancer entity revealed a series of cancer genes and biologically relevant subtype diversity that represent attractive therapeutic targets for the treatment of patients with medulloblastoma., Author(s): Paul A. Northcott [1, 2]; Ivo Buchhalter [3, 4, 5]; A. Sorana Morrissy [6]; Volker Hovestadt [7]; Joachim Weischenfeldt [8]; Tobias Ehrenberger [9]; Susanne Grbner [1, 10]; Maia Segura-Wang [...]

Published
2017
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24. Clinical cancer genomic profiling by three-platform sequencing of whole genome, whole exome and transcriptome

Author

Rusch, Michael, Nakitandwe, Joy, Shurtleff, Sheila, Newman, Scott, Zhang, Zhaojie, Edmonson, Michael N., Parker, Matthew, Jiao, Yuannian, Ma, Xiaotu, Liu, Yanling, Gu, Jiali, Walsh, Michael F., Becksfort, Jared, Thrasher, Andrew, Li, Yongjin, McMurry, James, Hedlund, Erin, Patel, Aman, Easton, John, Yergeau, Donald, Vadodaria, Bhavin, Tatevossian, Ruth G., Raimondi, Susana, Hedges, Dale, Chen, Xiang, Hagiwara, Kohei, McGee, Rose, Robinson, Giles W., Klco, Jeffery M., Gruber, Tanja A., Ellison, David W., Downing, James R, and Zhang, Jinghui

Published
2018
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25. Risk stratification of childhood medulloblastoma in the molecular era: the current consensus

Author

Ramaswamy, Vijay, Remke, Marc, Bouffet, Eric, Bailey, Simon, Clifford, Steven C., Doz, Francois, Kool, Marcel, Dufour, Christelle, Vassal, Gilles, Milde, Till, Witt, Olaf, von Hoff, Katja, Pietsch, Torsten, Northcott, Paul A., Gajjar, Amar, Robinson, Giles W., Padovani, Laetitia, André, Nicolas, Massimino, Maura, Pizer, Barry, Packer, Roger, Rutkowski, Stefan, Pfister, Stefan M., Taylor, Michael D., and Pomeroy, Scott L.

Published
2016
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27. A sellar presentation of a WNT-activated embryonal tumor: further evidence of an ectopic medulloblastoma.

Author

Tauziède-Espariat, Arnault, Simbozel, Marie, Liu, Anthony P. Y., Robinson, Giles W., Masliah-Planchon, Julien, Sievers, Philipp, Vasiljevic, Alexandre, Duchesne, Mathilde, Puget, Stéphanie, Dangouloff-Ros, Volodia, Boddaert, Nathalie, Métais, Alice, Hasty, Lauren, Dufour, Christelle, and Varlet, Pascale

Subjects
MEDULLOBLASTOMA, POSTERIOR cranial fossa, INFRATENTORIAL brain tumors
Abstract

However, a recent study reported 7 cases of WNT-activated embryonal tumors epicentered in the pineal region that clustered within the DNA-methylation class of medulloblastomas, WNT-activated (MB-WNT) [[6]]. Indeed, for this case, there was no tumor in the posterior fossa, and the first biopsy showed a non-tumoral cerebellar parenchyma which may constitute the medulloblastoma cellular origin. The first biopsy of the lesion showed an atrophic cerebellar parenchyma with Purkinje neurons and granular cells (Fig. H Reference DNA methylation classes: medulloblastomas, WNT-activated (MB WNT); medulloblastomas, SHH-activated (MB SHH); medulloblastomas, group 3 (MB G3); medulloblastomas, group 4 (MB G4); pineoblastomas (PB); previously reported embryonal tumors, WNT-activated of the pineal gland (WNT-PB); and the current case. [Extracted from the article]

Published
2023
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29. DNA-methylation subgroups carry no prognostic significance in ATRT-SHH patients in clinical trial cohorts.

Author

Tran, Quynh T., Upadhyaya, Santhosh A., Billups, Catherine A., Onar-Thomas, Arzu, Alom, Md. Zahangir, Carey, Steven S., Robinson, Giles W., Ellison, David W., Gajjar, Amar, and Orr, Brent A.

Subjects
METHYLGUANINE, CLINICAL trials, CENTRAL nervous system tumors
Abstract

Consensus subgroup labels were reliably assigned to 41/43 cases, with 14 designated ATRT-SHH-1A, 11 ATRT-SHH-1B, and 16 ATRT-SHH-2 (Table S1). We also confirmed the previously observed difference in age at diagnosis with the ATRT-SHH-1B patients presenting at a median age of 3.5 years, compared to 1.1 years for both ATRT-SHH-1A and ATRT-SHH-2 ( I p i = 0.008). [Extracted from the article]

Published
2023
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30. Entrectinib in children and young adults with solid or primary CNS tumors harboring NTRK, ROS1, or ALK aberrations (STARTRK-NG).

Author

Desai, Ami V, Robinson, Giles W, Gauvain, Karen, Basu, Ellen M, Macy, Margaret E, Maese, Luke, Whipple, Nicholas S, Sabnis, Amit J, Foster, Jennifer H, Shusterman, Suzanne, Yoon, Janet, Weiss, Brian D, Abdelbaki, Mohamed S, Armstrong, Amy E, Cash, Thomas, Pratilas, Christine A, Corradini, Nadège, Marshall, Lynley V, Farid-Kapadia, Mufiza, and Chohan, Saibah

Published
2022
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31. Enhancer hijacking activates GFI1 family oncogenes in medulloblastoma

Author

Northcott, Paul A., Lee, Catherine, Zichner, Thomas, Stütz, Adrian M., Erkek, Serap, Kawauchi, Daisuke, Shih, David J. H., Hovestadt, Volker, Zapatka, Marc, Sturm, Dominik, Jones, David T. W., Kool, Marcel, Remke, Marc, Cavalli, Florence M. G., Zuyderduyn, Scott, Bader, Gary D., VandenBerg, Scott, Esparza, Lourdes Adriana, Ryzhova, Marina, Wang, Wei, Wittmann, Andrea, Stark, Sebastian, Sieber, Laura, Seker-Cin, Huriye, Linke, Linda, Kratochwil, Fabian, Jäger, Natalie, Buchhalter, Ivo, Imbusch, Charles D., Zipprich, Gideon, Raeder, Benjamin, Schmidt, Sabine, Diessl, Nicolle, Wolf, Stephan, Wiemann, Stefan, Brors, Benedikt, Lawerenz, Chris, Eils, Jürgen, Warnatz, Hans-Jörg, Risch, Thomas, Yaspo, Marie-Laure, Weber, Ursula D., Bartholomae, Cynthia C., von Kalle, Christof, Turányi, Eszter, Hauser, Peter, Sanden, Emma, Darabi, Anna, Siesjö, Peter, Sterba, Jaroslav, Zitterbart, Karel, Sumerauer, David, van Sluis, Peter, Versteeg, Rogier, Volckmann, Richard, Koster, Jan, Schuhmann, Martin U., Ebinger, Martin, Grimes, Leighton H., Robinson, Giles W., Gajjar, Amar, Mynarek, Martin, von Hoff, Katja, Rutkowski, Stefan, Pietsch, Torsten, Scheurlen, Wolfram, Felsberg, Jörg, Reifenberger, Guido, Kulozik, Andreas E., von Deimling, Andreas, Witt, Olaf, Eils, Roland, Gilbertson, Richard J., Korshunov, Andrey, Taylor, Michael D., Lichter, Peter, Korbel, Jan O., Wechsler-Reya, Robert J., and Pfister, Stefan M.

Published
2014
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32. Revised clinical and molecular risk strata define the incidence and pattern of failure in medulloblastoma following risk-adapted radiotherapy and dose-intensive chemotherapy: results from a phase III multi-institutional study.

Author

Lucas, John T, Tinkle, Christopher L, Huang, Jie, Onar-Thomas, Arzu, Srinivasan, Sudharsan, Tumlin, Parker, Becksfort, Jared B, Klimo, Paul, Boop, Frederick A, Robinson, Giles W, Orr, Brent A, Harreld, Julie H, Krasin, Matthew J, Northcott, Paul A, Ellison, David W, Gajjar, Amar, and Merchant, Thomas E

Published
2022
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37. Clinical Outcomes and Patient-Matched Molecular Composition of Relapsed Medulloblastoma.

Author

Kumar, Rahul, Smith, Kyle S, Deng, Maximilian, Terhune, Colt, Robinson, Giles W, Orr, Brent A, Liu, Anthony P Y, Lin, Tong, Billups, Catherine A, Chintagumpala, Murali, Bowers, Daniel C, Hassall, Timothy E, Hansford, Jordan R, Khuong-Quang, Dong Anh, Crawford, John R, Bendel, Anne E, Gururangan, Sridharan, Schroeder, Kristin, Bouffet, Eric, and Bartels, Ute

Published
2021
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38. Outcomes by Clinical and Molecular Features in Children With Medulloblastoma Treated With Risk-Adapted Therapy: Results of an International Phase III Trial (SJMB03).

Author

Gajjar, Amar, Robinson, Giles W, Smith, Kyle S, Lin, Tong, Merchant, Thomas E, Chintagumpala, Murali, Mahajan, Anita, Su, Jack, Bouffet, Eric, Bartels, Ute, Schechter, Tal, Hassall, Tim, Robertson, Thomas, Nicholls, Wayne, Gururangan, Sridharan, Schroeder, Kristin, Sullivan, Michael, Wheeler, Greg, Hansford, Jordan R, and Kellie, Stewart J

Published
2021
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40. Phase II study of peginterferon alpha-2b for patients with unresectable or recurrent craniopharyngiomas: a Pediatric Brain Tumor Consortium report.

Author

Goldman, Stewart, Pollack, Ian F, Jakacki, Regina I, Billups, Catherine A, Poussaint, Tina Y, Adesina, Adekunle M, Panigrahy, Ashok, Parsons, Donald W, Broniscer, Alberto, Robinson, Giles W, Robison, Nathan J, Partap, Sonia, Kilburn, Lindsay B, Onar-Thomas, Arzu, Dunkel, Ira J, and Fouladi, Maryam

Published
2020
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43. Germline Mutations Predispose to Pediatric Medulloblastoma.

Author

Begemann, Matthias, Waszak, Sebastian M., Robinson, Giles W., Jäger, Natalie, Sharma, Tanvi, Knopp, Cordula, Kraft, Florian, Moser, Olga, Mynarek, Martin, Guerrini-Rousseau, Lea, Brugieres, Laurence, Varlet, Pascale, Pietsch, Torsten, Bowers, Daniel C., Chintagumpala, Murali, Sahm, Felix, Korbel, Jan O., Rutkowski, Stefan, Eggermann, Thomas, and Gajjar, Amar

Published
2020
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44. Molecular grouping and outcomes of young children with newly diagnosed ependymoma treated on the multi-institutional SJYC07 trial.

Author

Upadhyaya, Santhosh A, Robinson, Giles W, Onar-Thomas, Arzu, Orr, Brent A, Billups, Catherine A, Bowers, Daniel C, Bendel, Anne E, Hassall, Tim, Crawford, John R, Partap, Sonia, Fisher, Paul G, Tatevossian, Ruth G, Seah, Tiffany, Qaddoumi, Ibrahim A, Vinitsky, Anna, Armstrong, Gregory T, Sabin, Noah D, Tinkle, Christopher L, Klimo, Paul, and Indelicato, Danny J

Published
2019
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46. Advances in the classification of pediatric brain tumors through DNA methylation profiling: From research tool to frontline diagnostic.

Author

Kumar, Rahul, Liu, Anthony P. Y., Orr, Brent A., Northcott, Paul A., and Robinson, Giles W.

Subjects
EPIGENETICS, MOLECULAR pathology, CENTRAL nervous system tumors, DNA methylation, EMBRYONAL tumors, MEDULLOBLASTOMA
Abstract

Despite significant improvements in pediatric brain tumor therapy and outcome, too many children still die of disease, and too many survivors experience significant sequelae as a result of conventional therapies. The molecular characterization of pediatric brain tumors has afforded tremendous insight into the basic biology and clinical management of these deadly childhood diseases. Genomic, epigenomic, and transcriptional profiling have facilitated the identification of significant heterogeneity among previously uniform disease entities. In particular, DNA methylation profiling has emerged as a robust tool for identifying key disease-specific subgroups that can exhibit distinct clinical outcomes. These approaches, which also complement classic histologic techniques, can suggest key mechanistic underpinnings of tumorigenesis and open the door for better informed and more tailored therapy. By leveraging the results of large-scale classifications of disease cohorts, novel driver mutations and pathways can be uncovered, enabling the generation of faithful animal models, promoting targeted drug design, informing developmental biology, and ultimately translating into improved clinical management. In this review, progress in the epigenetic classification of common malignant pediatric brain tumors, namely medulloblastoma, ependymoma, high-grade glioma, atypical teratoid/rhabdoid tumor, and central nervous system embryonal tumors, will be discussed, and the potential role of DNA methylation profiling as a frontline diagnostic modality will be emphasized. [ABSTRACT FROM AUTHOR]

Published
2018
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47. WNT-activated embryonal tumors of the pineal region: ectopic medulloblastomas or a novel pineoblastoma subgroup?

Author

Liu, Anthony P. Y., Priesterbach-Ackley, Loudy P., Orr, Brent A., Li, Bryan K., Gudenas, Brian, Reddingius, Roel E., Suñol, Mariona, Lavarino, Cinzia E., Olaciregui, Nagore G., Santa-María López, Vicente, Fisher, Michael J., Hazrati, Lili-Naz, Bouffet, Eric, Huang, Annie, Robinson, Giles W., Wesseling, Pieter, Northcott, Paul A., and Gajjar, Amar

Subjects
TUMORS, NEURAL stem cells
Published
2020
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50. Risk-adapted therapy for young children with medulloblastoma (SJYC07): therapeutic and molecular outcomes from a multicentre, phase 2 trial.

Author

Robinson, Giles W, Rudneva, Vasilisa A, Buchhalter, Ivo, Billups, Catherine A, Waszak, Sebastian M, Smith, Kyle S, Bowers, Daniel C, Bendel, Anne, Fisher, Paul G, Partap, Sonia, Crawford, John R, Hassall, Tim, Indelicato, Daniel J, Boop, Frederick, Klimo, Paul, Sabin, Noah D, Patay, Zoltan, Merchant, Thomas E, Stewart, Clinton F, and Orr, Brent A

Subjects
*MEDULLOBLASTOMA, *CHILDHOOD cancer, *CANCER radiotherapy, *PROGRESSION-free survival, *CANCER chemotherapy, *THERAPEUTICS, *BRAIN tumor treatment, *GLIOMA treatment, *AGE distribution, *ANTINEOPLASTIC agents, *BRAIN tumors, *COMBINED modality therapy, *COMPARATIVE studies, *GLIOMAS, *RESEARCH methodology, *MEDICAL cooperation, *RADIATION doses, *RADIOTHERAPY, *RESEARCH, *RESEARCH funding, *RISK assessment, *TIME, *EVALUATION research, *PREDICTIVE tests, *PATIENT selection, *DNA methylation, *GENE expression profiling
Abstract

Background: Young children with medulloblastoma have a poor overall survival compared with older children, due to use of radiation-sparing therapy in young children. Radiotherapy is omitted or reduced in these young patients to spare them from debilitating long-term side-effects. We aimed to estimate event-free survival and define the molecular characteristics associated with progression-free survival in young patients with medulloblastoma using a risk-stratified treatment strategy designed to defer, reduce, or delay radiation exposure.Methods: In this multicentre, phase 2 trial, we enrolled children younger than 3 years with newly diagnosed medulloblastoma at six centres in the USA and Australia. Children aged 3-5 years with newly diagnosed, non-metastatic medulloblastoma without any high-risk features were also eligible. Eligible patients were required to start therapy within 31 days from definitive surgery, had a Lansky performance score of at least 30, and did not receive previous radiotherapy or chemotherapy. Patients were stratified postoperatively by clinical and histological criteria into low-risk, intermediate-risk, and high-risk treatment groups. All patients received identical induction chemotherapy (methotrexate, vincristine, cisplatin, and cyclophosphamide), with high-risk patients also receiving an additional five doses of vinblastine. Induction was followed by risk-adapted consolidation therapy: low-risk patients received cyclophosphamide (1500 mg/m2 on day 1), etoposide (100 mg/m2 on days 1 and 2), and carboplatin (area under the curve 5 mg/mL per min on day 2) for two 4-week cycles; intermediate-risk patients received focal radiation therapy (54 Gy with a clinical target volume of 5 mm over 6 weeks) to the tumour bed; and high-risk patients received chemotherapy with targeted intravenous topotecan (area under the curve 120-160 ng-h/mL intravenously on days 1-5) and cyclophosphamide (600 mg/m2 intravenously on days 1-5). After consolidation, all patients received maintenance chemotherapy with cyclophosphamide, topotecan, and erlotinib. The coprimary endpoints were event-free survival and patterns of methylation profiling associated with progression-free survival. Outcome and safety analyses were per protocol (all patients who received at least one dose of induction chemotherapy); biological analyses included all patients with tissue available for methylation profiling. This trial is registered with ClinicalTrials.gov, number NCT00602667, and was closed to accrual on April 19, 2017.Findings: Between Nov 27, 2007, and April 19, 2017, we enrolled 81 patients with histologically confirmed medulloblastoma. Accrual to the low-risk group was suspended after an interim analysis on Dec 2, 2015, when the 1-year event-free survival was estimated to be below the stopping rule boundary. After a median follow-up of 5·5 years (IQR 2·7-7·3), 5-year event-free survival was 31·3% (95% CI 19·3-43·3) for the whole cohort, 55·3% (95% CI 33·3-77·3) in the low-risk cohort (n=23) versus 24·6% (3·6-45·6) in the intermediate-risk cohort (n=32; hazard ratio 2·50, 95% CI 1·19-5·27; p=0·016) and 16·7% (3·4-30·0) in the high-risk cohort (n=26; 3·55, 1·66-7·59; p=0·0011; overall p=0·0021). 5-year progression-free survival by methylation subgroup was 51·1% (95% CI 34·6-67·6) in the sonic hedgehog (SHH) subgroup (n=42), 8·3% (95% CI 0·0-24·0%) in the group 3 subgroup (n=24), and 13·3% (95% CI 0·0-37·6%) in the group 4 subgroup (n=10). Within the SHH subgroup, two distinct methylation subtypes were identified and named iSHH-I and iSHH-II. 5-year progression-free survival was 27·8% (95% CI 9·0-46·6; n=21) for iSHH-I and 75·4% (55·0-95·8; n=21) for iSHH-II. The most common adverse events were grade 3-4 febrile neutropenia (48 patients [59%]), neutropenia (21 [26%]), infection with neutropenia (20 [25%]), leucopenia (15 [19%]), vomiting (15 [19%]), and anorexia (13 [16%]). No treatment-related deaths occurred.Interpretation: The risk-adapted approach did not improve event-free survival in young children with medulloblastoma. However, the methylation subgroup analyses showed that the SHH subgroup had improved progression-free survival compared with the group 3 subgroup. Moreover, within the SHH subgroup, the iSHH-II subtype had improved progression-free survival in the absence of radiation, intraventricular chemotherapy, or high-dose chemotherapy compared with the iSHH-I subtype. These findings support the development of a molecularly driven, risk-adapted, treatment approach in future trials in young children with medulloblastoma.Funding: American Lebanese Syrian Associated Charities, St Jude Children's Research Hospital, NCI Cancer Center, Alexander and Margaret Stewart Trust, Sontag Foundation, and American Association for Cancer Research. [ABSTRACT FROM AUTHOR]

Published
2018
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