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183


Novel Candidate Oncogenic Drivers in Pineoblastoma [Meeting Abstract]

Snuderl, Matija; Kannan, Kasthuri; Aminova, Olga; Dolgalev, Igor; Heguy, Adriana; Faustin, Arline; Zagzag, David; Gardner, Sharon; Allen, Jeffrey; Wisoff, Jeffrey; Capper, David; Hovestadt, Volker; Ahsan, Sama; Eberhart, Charles; Pfister, Stefan; Jones, David; Karajannis, Matthias
ISI:000354824800135
ISSN: 0022-3069
CID: 1620172

Genomic and Epigenetic Landscape of Sudden Unexpected Death in Epilepsy [Meeting Abstract]

Faustin, Arline; Kannan, Kasthuri; Friedman, Daniel; Shroff, Seema; Thomas, Cheddhi; Karajannis, Matthias; Heguy, Adriana; Serrano, Jonathan; Wisniewski, Thomas; Zagzag, David; Devinsky, Orrin; Snuderl, Matija
ISI:000354824800017
ISSN: 0022-3069
CID: 1620452

Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups

Pajtler, Kristian W; Witt, Hendrik; Sill, Martin; Jones, David T W; Hovestadt, Volker; Kratochwil, Fabian; Wani, Khalida; Tatevossian, Ruth; Punchihewa, Chandanamali; Johann, Pascal; Reimand, Juri; Warnatz, Hans-Jorg; Ryzhova, Marina; Mack, Steve; Ramaswamy, Vijay; Capper, David; Schweizer, Leonille; Sieber, Laura; Wittmann, Andrea; Huang, Zhiqin; van Sluis, Peter; Volckmann, Richard; Koster, Jan; Versteeg, Rogier; Fults, Daniel; Toledano, Helen; Avigad, Smadar; Hoffman, Lindsey M; Donson, Andrew M; Foreman, Nicholas; Hewer, Ekkehard; Zitterbart, Karel; Gilbert, Mark; Armstrong, Terri S; Gupta, Nalin; Allen, Jeffrey C; Karajannis, Matthias A; Zagzag, David; Hasselblatt, Martin; Kulozik, Andreas E; Witt, Olaf; Collins, V Peter; von Hoff, Katja; Rutkowski, Stefan; Pietsch, Torsten; Bader, Gary; Yaspo, Marie-Laure; von Deimling, Andreas; Lichter, Peter; Taylor, Michael D; Gilbertson, Richard; Ellison, David W; Aldape, Kenneth; Korshunov, Andrey; Kool, Marcel; Pfister, Stefan M
Ependymal tumors across age groups are currently classified and graded solely by histopathology. It is, however, commonly accepted that this classification scheme has limited clinical utility based on its lack of reproducibility in predicting patients' outcome. We aimed at establishing a uniform molecular classification using DNA methylation profiling. Nine molecular subgroups were identified in a large cohort of 500 tumors, 3 in each anatomical compartment of the CNS, spine, posterior fossa, supratentorial. Two supratentorial subgroups are characterized by prototypic fusion genes involving RELA and YAP1, respectively. Regarding clinical associations, the molecular classification proposed herein outperforms the current histopathological classification and thus might serve as a basis for the next World Health Organization classification of CNS tumors.
PMCID:4712639
PMID: 25965575
ISSN: 1878-3686
CID: 1578762

Neurofibromatosis-related tumors: emerging biology and therapies

Karajannis, Matthias A; Ferner, Rosalie E
PURPOSE OF REVIEW: Over the past decade, substantial insight into the biological function of the tumor suppressors neurofibromin (NF1) and Merlin (NF2) has been gained. The purpose of this review is to highlight some of the major advances in our understanding of the biology of neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2) as they relate to the development of novel therapies for these disorders. RECENT FINDINGS: The development of increasingly sophisticated preclinical models over the recent years has provided the platform from which to rationally develop molecular targeted therapies for both NF1 and NF2-related tumors, such as within the Department of Defense-sponsored Neurofibromatosis Clinical Trials Consortium. SUMMARY: Clinical trials with molecular-targeted therapies have become a reality for neurofibromatosis patients, and hold substantial promise for improving the morbidity and mortality of individuals affected with these disorders.
PMCID:4374132
PMID: 25490687
ISSN: 1040-8703
CID: 1459772

Schwannomas

Chapter by: Karajannis, Matthias A; Stemmer-Rachamimov, Anat
in: MOLECULAR PATHOLOGY OF NERVOUS SYSTEM TUMORS: BIOLOGICAL STRATIFICATION AND TARGETED THERAPIES by Karajannis, MA; Zagzag, D [Eds]
NEW YORK : SPRINGER, 2015
pp. 201-211
ISBN:
CID: 2658812

Hereditary Predisposition to Primary CNS Tumors

Chapter by: Tabori, Uri; Karajannis, Matthias A; Pappas, John G
in: MOLECULAR PATHOLOGY OF NERVOUS SYSTEM TUMORS: BIOLOGICAL STRATIFICATION AND TARGETED THERAPIES by Karajannis, MA; Zagzag, D [Eds]
NEW YORK : SPRINGER, 2015
pp. 1-22
ISBN:
CID: 2658802

Novel candidate oncogenic drivers in pineoblastoma [Meeting Abstract]

Snuderl, Matija; Kannan, Kasthuri; Aminova, Olga; Dolgalev, Igor; Heguy, Adriana; Faustin, Arline; Zagzag, David; Gardner, Sharon L; Anen, Jeffrey C; Wisoff, Jeffrey H; Capper, David; Hovestadt, Volker; Ahsan, Sama; Eberhart, Charles; Pfister, Stefan M; Jones, David TW; Karajannis, Matthias A
ISI:000371597100272
ISSN: 1538-7445
CID: 2064382

Clinical response to bevacizumab in schwannomatosis

Blakeley, Jaishri; Schreck, Karisa C; Evans, D Gareth; Korf, Bruce R; Zagzag, David; Karajannis, Matthias A; Bergner, Amanda L; Belzberg, Allan J
PMCID:4248457
PMID: 25339217
ISSN: 0028-3878
CID: 1368962

Recurrent Ascites in a Patient With Low-grade Astrocytoma and Ventriculo-Peritoneal Shunt Treated With the Multikinase Inhibitor Sorafenib

Legault, Genevieve; Kieran, Mark W; Scott, Robert Michael; Chordas, Christine; Milla, Sarah S; Karajannis, Matthias A
This report describes a 6-year-old boy with disseminated low-grade astrocytoma and ventriculo-peritoneal shunt, who developed recurrent ascites while receiving sorafenib on a clinical trial. Laboratory analysis of the peritoneal fluid showed no elevation of protein content and no evidence of underlying infection or tumor dissemination. This report highlights ascites as a previously unrecognized adverse reaction to sorafenib in a patient with a ventriculo-peritoneal shunt. We conclude that such patients should be closely monitored for this complication when treated with sorafenib.
PMID: 24351969
ISSN: 1077-4114
CID: 760302

Next-generation neuropathology - Improving diagnostic accuracy for brain tumors using DNA methylation array-based molecular profiling [Meeting Abstract]

Jones, David TW; Capper, David; Sill, Martin; Hovestadt, Volker; Schweizer, Leonille; Fischer, Roger; Schick, Matthias; Bewerunge-Hudler, Melanie; Benner, Axel; Zagzag, David; Lichter, Peter; Karajannis, Matthias A; Aldape, Kenneth D; Korshunov, Andrey; von Deimling, Andreas; Pfister, Stefan M
ISI:000349906902106
ISSN: 1538-7445
CID: 1599132