Now showing items 1-20 of 44

    • Achieving consensus for clinical trials: The REiNS International Collaboration 

      Plotkin, Scott Randall; Blakeley, J. O.; Dombi, Eva; Fisher, Michael J.; Hanemann, C. O.; Walsh, Karin S; Wolters, P. L.; Widemann, Brigitte C. (Ovid Technologies (Wolters Kluwer Health), 2013)
      The neurofibromatoses (NF)—including neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), and schwannomatosis—are related tumor-suppressor syndromes characterized by a predisposition to multiple tumor types and other ...
    • Anti-Vascular Endothelial Growth Factor Therapies as a Novel Therapeutic Approach to Treating Neurofibromatosis-Related Tumors 

      Wong, H. K.; Lahdenranta, J.; Kamoun, W. S.; Chan, Ariel Hin Man; McClatchey, Andrea I.; Plotkin, Scott Randall; Jain, Rakesh K.; di Tomaso, E. (American Association for Cancer Research (AACR), 2010)
      Patients with bilateral vestibular schwannomas associated with neurofibromatosis type 2 (NF2) experience significant morbidity such as complete hearing loss. We have recently shown that treatment with bevacizumab provided ...
    • AZD2171, a Pan-VEGF Receptor Tyrosine Kinase Inhibitor, Normalizes Tumor Vasculature and Alleviates Edema in Glioblastoma Patients 

      Batchelor, Tracy Todd; Sorensen, A; di Tomaso, Emmanuelle; Zhang, Wei-Ting; Duda, Dan Gabriel; Cohen, Kenneth S.; Kozak, Kevin R.; Cahill, Daniel P.; Chen, Poe-Jou; Zhu, Mingwang; Ancukiewicz, Marek; Mrugala, Maciej M.; Plotkin, Scott Randall; Drappatz, Jan; Louis, David N.; Ivy, Percy; Scadden, David Thomas; Benner, Thomas; Loeffler, Jay Steven; Wen, Patrick Yung Chih; Jain, Rakesh K. (Elsevier BV, 2007)
      Using MRI techniques, we show here that normalization of tumor vessels in recurrent glioblastoma patients by daily administration of AZD2171—an oral tyrosine kinase inhibitor of VEGF receptors—has rapid onset, is prolonged ...
    • Bevacizumab for Progressive Vestibular Schwannoma in Neurofibromatosis Type 2 

      Plotkin, Scott Randall; Merker, Vanessa; Halpin, Chris; Jennings, Dominique; McKenna, Michael John; Harris, Gordon J.; Barker, Frederick George (Ovid Technologies (Wolters Kluwer Health), 2012)
      Objective: Early studies suggest that bevacizumab treatment can result in tumor shrinkage and hearing improvement for some patients with neurofibromatosis type 2 (NF2). The aim of this study was to report extended follow-up ...
    • Bevacizumab Treatment for Meningiomas in NF2: A Retrospective Analysis of 15 Patients 

      Nunes, Fabio; Merker, Vanessa L.; Jennings, Dominique; Caruso, Paul Albert; di Tomaso, Emmanuelle; Muzikansky, Alona; Barker, Frederick George; Stemmer-Rachamimov, Anat; Plotkin, Scott Randall (Public Library of Science (PLoS), 2013)
      Bevacizumab treatment can result in tumor shrinkage of progressive vestibular schwannomas in some neurofibromatosis 2 (NF2) patients but its effect on meningiomas has not been defined. To determine the clinical activity ...
    • Bevacizumab treatment for symptomatic spinal ependymomas in neurofibromatosis type 2 

      Farschtschi, S.; Merker, V. L.; Wolf, D.; Schuhmann, M.; Blakeley, J.; Plotkin, Scott Randall; Hagel, C.; Mautner, V. F. (Wiley-Blackwell, 2015)
      Background Neurofibromatosis type 2 (NF2) is a tumor suppressor syndrome associated with vestibular schwannomas, meningiomas, and spinal ependymomas. There have been anecdotal reports of radiographic response of spinal ...
    • Clinical Features of Schwannomatosis: A Retrospective Analysis of 87 Patients 

      Merker, Vanessa; Esparza, S.; Smith, M. J.; Stemmer-Rachamimov, Anat; Plotkin, Scott Randall (Alphamed Press, 2012)
      Background. Schwannomatosis is a recently recognized form of neurofibromatosis characterized by multiple noncutaneous schwannomas, a histologically benign nerve sheath tumor. As more cases are identified, the reported ...
    • Clinical Reasoning: A case of multiple intracerebral hemorrhages 

      Wendell, L. C.; Freeman, S. H.; Plotkin, Scott Randall; Sims, John R (Ovid Technologies (Wolters Kluwer Health), 2007)
    • CTF meeting 2012: Translation of the basic understanding of the biology and genetics of NF1, NF2, and schwannomatosis toward the development of effective therapies 

      Widemann, Brigitte C.; Acosta, Maria T.; Ammoun, Sylvia; Belzberg, Allan J.; Bernards, Andre; Blakeley, Jaishri; Bretscher, Antony; Cichowski, Karen Marie; Clapp, D. Wade; Dombi, Eva; Evans, Gareth D.; Ferner, Rosalie; Fernandez-Valle, Cristina; Fisher, Michael J.; Giovannini, Marco; Gutmann, David H.; Hanemann, C. Oliver; Hennigan, Robert; Huson, Susan; Ingram, David; Kissil, Joe; Korf, Bruce R.; Legius, Eric; Packer, Roger J.; McClatchey, Andrea I.; McCormick, Frank; North, Kathryn; Pehrsson, Minja; Plotkin, Scott Randall; Ramesh, Vijaya; Ratner, Nancy; Schirmer, Susann; Sherman, Larry; Schorry, Elizabeth; Stevenson, David; Stewart, Douglas C; Ullrich, Nicole Johnson; Bakker, Annette C.; Morrison, Helen (Wiley-Blackwell, 2014)
      The neurofibromatoses (NF) are autosomal dominant genetic disorders that encompass the rare diseases NF1, NF2, and schwannomatosis. The NFs affect more people worldwide than Duchenne muscular dystrophy and Huntington's ...
    • Current status and recommendations for biomarkers and biobanking in neurofibromatosis 

      Hanemann, C. Oliver; Blakeley, Jaishri O.; Nunes, Fabio; Robertson, Kent; Stemmer-Rachamimov, Anat; Mautner, Victor; Kurtz, Andreas; Ferguson, Michael Andrew; Widemann, Brigitte C.; Evans, D. Gareth; Ferner, Rosalie; Carroll, Steven L.; Korf, Bruce; Wolkenstein, Pierre; Knight, Pamela; Plotkin, Scott Randall (Ovid Technologies (Wolters Kluwer Health), 2016)
      Objective: Clinically validated biomarkers for neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), and schwannomatosis (SWN) have not been identified to date. The biomarker working group’s goals are to (1) define ...
    • Efficacy and Biomarker Study of Bevacizumab for Hearing Loss Resulting From Neurofibromatosis Type 2–Associated Vestibular Schwannomas 

      Blakeley, Jaishri O.; Ye, Xiaobu; Duda, Dan Gabriel; Halpin, Chris F.; Bergner, Amanda L.; Muzikansky, Alona; Merker, Vanessa; Gerstner, Elizabeth Robins; Fayad, Laura M.; Ahlawat, Shivani; Jacobs, Michael A.; Jain, Rakesh K.; Zalewski, Christopher; Dombi, Eva; Widemann, Brigitte C.; Plotkin, Scott Randall (American Society of Clinical Oncology (ASCO), 2016)
      Purpose Neurofibromatosis type 2 (NF2) is a tumor predisposition syndrome characterized by bilateral vestibular schwannomas (VSs) resulting in deafness and brainstem compression. This study evaluated efficacy and ...
    • Emotional functioning of patients with neurofibromatosis tumor suppressor syndrome 

      Wang, Daphne L.; Smith, Kelly; Esparza, Sonia; Leigh, Fawn; Muzikansky, Alona; Park, Elyse Richelle; Plotkin, Scott Randall (Springer Nature, 2012)
      Purpose Although patients with neurofibromatosis are predisposed to multiple nerve sheath tumors that can develop anywhere in the body and cause significant morbidity (e.g., hearing loss; pain), little research has ...
    • Erlotinib for Progressive Vestibular Schwannoma in Neurofibromatosis 2 Patients 

      Plotkin, Scott Randall; Halpin, Chris; McKenna, Michael; Loeffler, Jay Steven; Batchelor, Tracy Todd; Barker, Frederick George (Ovid Technologies (Wolters Kluwer Health), 2010)
      In vitro treatment of Nf2-deficient cells with epidermal growth factor receptor (EGFR) inhibitors can inhibit cellular proliferation. We retrospectively assessed the effect of erlotinib (150 mg daily) on eleven consecutive ...
    • The Fourth International Symposium on Genetic Disorders of the Ras/MAPK pathway 

      Stevenson, David A.; Schill, Lisa; Schoyer, Lisa; Andresen, Brage S.; Bakker, Annette; Bayrak-Toydemir, Pinar; Burkitt-Wright, Emma; Chatfield, Kathryn; Elefteriou, Florent; Elgersma, Ype; Fisher, Michael J.; Franz, David; Gelb, Bruce D.; Goriely, Anne; Gripp, Karen W.; Hardan, Antonio Y.; Keppler-Noreuil, Kim M.; Kerr, Bronwyn; Korf, Bruce; Leoni, Chiara; McCormick, Frank; Plotkin, Scott Randall; Rauen, Katherine A.; Reilly, Karlyne; Roberts, Amy Elizabeth; Sandler, Abby; Siegel, Dawn; Walsh, Karin; Widemann, Brigitte C. (Wiley-Blackwell, 2016)
      The RASopathies are a group of disorders due to variations of genes associated with the Ras/MAPK pathway. Some of the RASopathies include neurofibromatosis type 1 (NF1), Noonan syndrome, Noonan syndrome with multiple ...
    • Genomic profiling distinguishes familial multiple and sporadic multiple meningiomas 

      Engler, David A; Roy, Jennifer; Shen, Yiping; Nunes, Fabio Pereira; Stemmer-Rachamimov, Anat; James, Marianne F.; Mohapatra, Gayatry; Plotkin, Scott Randall; Betensky, Rebecca Aubrey; Ramesh, Vijaya; Gusella, James Francis (BioMed Central, 2009)
      Background: Meningiomas may occur either as familial tumors in two distinct disorders, familial multiple meningioma and neurofibromatosis 2 (NF2), or sporadically, as either single or multiple tumors in individuals with ...
    • Glioblastoma Recurrence after Cediranib Therapy in Patients: Lack of "Rebound" Revascularization as Mode of Escape 

      di Tomaso, E.; Snuderl, M.; Kamoun, W. S.; Duda, Dan Gabriel; Auluck, P. K.; Fazlollahi, L.; Andronesi, Ovidiu C; Frosch, Matthew P.; Wen, Patrick Yung Chih; Plotkin, Scott Randall; Hedley-Whyte, E. Tessa; Sorensen, A; Batchelor, Tracy Todd; Jain, Radhika (American Association for Cancer Research (AACR), 2011)
      Recurrent glioblastomas (rGBM) invariably relapse after initial response to anti-VEGF therapy. There are two prevailing hypotheses on how these tumors escape antiangiogenic therapy: switch to VEGF-independent angiogenic ...
    • Health-related Quality of Life of Individuals With Neurofibromatosis Type 2 

      Merker, Vanessa; Bergner, Amanda L.; Vranceanu, Ana-Maria; Muzikansky, Alona; Slattery, William; Plotkin, Scott Randall (Ovid Technologies (Wolters Kluwer Health), 2016)
      Objective: To explore health-related quality of life (HRQoL) reported by individuals with neurofibromatosis type 2 (NF2) and to assess for correlations between HRQoL and objective measures of disease manifestations. Study ...
    • Hearing and facial function outcomes for neurofibromatosis 2 clinical trials 

      Plotkin, Scott Randall; Ardern-Holmes, S. L.; Barker, Frederick George; Blakeley, Jaishri O.; Evans, D. G.; Ferner, R. E.; Hadlock, Theresa A; Halpin, C. (Ovid Technologies (Wolters Kluwer Health), 2013)
      Objectives: Vestibular schwannomas are the hallmark of neurofibromatosis 2 (NF2), occurring in >95% of patients. These tumors develop on the vestibulocochlear nerve and are associated with significant morbidity due to ...
    • Hearing Improvement after Bevacizumab in Patients with Neurofibromatosis Type 2 

      Plotkin, Scott Randall; Stemmer-Rachamimov, Anat; Barker, Frederick George; Halpin, Chris; Padera, Timothy P.; Tyrrell, Alex; Sorensen, A; Jain, Rakesh K.; di Tomaso, Emmanuelle (New England Journal of Medicine (NEJM/MMS), 2009)
      Background Profound hearing loss is a serious complication of neurofibromatosis type 2, a genetic condition associated with bilateral vestibular schwannomas, benign tumors that arise from the eighth cranial nerve. There ...
    • A high-throughput kinome screen reveals serum/glucocorticoid-regulated kinase 1 as a therapeutic target for NF2-deficient meningiomas 

      Beauchamp, Roberta L.; James, Marianne; DeSouza, Patrick A.; Wagh, Vilas; Zhao, Wen-Ning; Jordan, Justin Thomas; Stemmer-Rachamimov, Anat; Plotkin, Scott Randall; Gusella, James Francis; Haggarty, Stephen John; Ramesh, Vijaya (Impact Journals, LLC, 2015)
      Meningiomas are the most common primary intracranial adult tumor. All Neurofibromatosis 2 (NF2)-associated meningiomas and ~60% of sporadic meningiomas show loss of NF2 tumor suppressor protein. There are no effective ...