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Correlation between NF1 genotype and imaging phenotype on whole-body MRI: NF1 radiogenomics.

Yunpeng Liu, Justin T Jordan, Miriam A Bredella, Serkan Erdin, James A Walker, Mark Vangel, Gordon J Harris, Scott R Plotkin, Wenli Cai,

OBJECTIVE:To investigate the genotype-phenotype correlation between neurofibromatosis 1 (NF1) germline mutations and imaging features of neurofibromas on whole-body MRI (WBMRI) by using radiomics image analysis techniques. MATERIALS AND METHODS:Twenty-nine patients with NF1 who had known germline mutations determined by targeted next-generation sequencing were selected from a previous WBMRI study using ... Read more >>

Neurology (Neurology)
[2020, 94(24):e2521-e2531]

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The Use of MEK Inhibitors in Neurofibromatosis Type 1-Associated Tumors and Management of Toxicities.

Laura J Klesse, Justin T Jordan, Heather B Radtke, Tena Rosser, Elizabeth Schorry, Nicole Ullrich, David Viskochil, Pamela Knight, Scott R Plotkin, Kaleb Yohay,

Early-phase clinical trials using oral inhibitors of MEK, the mitogen-activated protein kinase kinase, have demonstrated benefit for patients with neurofibromatosis type 1 (NF1)-associated tumors, particularly progressive low-grade gliomas and plexiform neurofibromas. Given this potential of MEK inhibition as an effective medical therapy, the use of targeted agents in the NF1 ... Read more >>

Oncologist (The oncologist)
[2020, :]

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Bevacizumab Reduces Permeability and Concurrent Temozolomide Delivery in a Subset of Patients with Recurrent Glioblastoma.

Elizabeth R Gerstner, Kyrre E Emblem, Ken Chang, Bella Vakulenko-Lagun, Yi-Fen Yen, Andrew L Beers, Jorg Dietrich, Scott R Plotkin, Ciprian Catana, Jacob M Hooker, Dan G Duda, Bruce Rosen, Jayashree Kalpathy-Cramer, Rakesh K Jain, Tracy Batchelor,

PURPOSE:Targeting tumor blood vessels is an attractive therapy in glioblastoma (GBM), but the mechanism of action of these agents and how they modulate delivery of concomitant chemotherapy are not clear in humans. We sought to elucidate how bevacizumab modulates tumor vasculature and the impact those vascular changes have on drug ... Read more >>

Clin. Cancer Res. (Clinical cancer research : an official journal of the American Association for Cancer Research)
[2020, 26(1):206-212]

Cited: 1 time

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Multicenter, Prospective, Phase II and Biomarker Study of High-Dose Bevacizumab as Induction Therapy in Patients With Neurofibromatosis Type 2 and Progressive Vestibular Schwannoma.

Scott R Plotkin, Dan G Duda, Alona Muzikansky, Jeffrey Allen, Jaishri Blakeley, Tena Rosser, Jian L Campian, D Wade Clapp, Michael J Fisher, James Tonsgard, Nicole Ullrich, Coretta Thomas, Gary Cutter, Bruce Korf, Roger Packer, Matthias A Karajannis,

PURPOSE:Bevacizumab treatment at 7.5 mg/kg every 3 weeks results in improved hearing in approximately 35%-40% of patients with neurofibromatosis type 2 (NF2) and progressive vestibular schwannomas (VSs). However, the optimal dose is unknown. In this multicenter phase II and biomarker study, we evaluated the efficacy and safety of high-dose bevacizumab ... Read more >>

J. Clin. Oncol. (Journal of clinical oncology : official journal of the American Society of Clinical Oncology)
[2019, 37(35):3446-3454]

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Publisher Correction: Probing tumor microenvironment in patients with newly diagnosed glioblastoma during chemoradiation and adjuvant temozolomide with functional MRI.

K Ina Ly, Bella Vakulenko-Lagun, Kyrre E Emblem, Yangming Ou, Xiao Da, Rebecca A Betensky, Jayashree Kalpathy-Cramer, Dan G Duda, Rakesh K Jain, Andrew S Chi, Scott R Plotkin, Tracy T Batchelor, Gregory Sorensen, Bruce R Rosen, Elizabeth R Gerstner,

A correction to this article has been published and is linked from the HTML and PDF versions of this paper. The error has been fixed in the paper. ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):8721]

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First report of quality of life in adults with neurofibromatosis 2 who are deafened or have significant hearing loss: results of a live-video randomized control trial.

Christopher J Funes, Ryan A Mace, Erik A Macklin, Scott R Plotkin, Justin T Jordan, Ana-Maria Vranceanu,

PURPOSE:To test the feasibility, acceptability, and preliminary efficacy of a mind-body program for patients with neurofibromatosis 2 (NF2) who are deaf or have significant hearing loss (d3RP-NF2) against an attention placebo control (dHEP-NF2) in a single-blind randomized control trial. Both were delivered using Communication Access Real-Time Translation and live group ... Read more >>

J. Neurooncol. (Journal of neuro-oncology)
[2019, 143(3):505-513]

Cited: 1 time

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First International Conference on RASopathies and Neurofibromatoses in Asia: Identification and advances of new therapeutics.

Katherine A Rauen, Abeer Alsaegh, Shay Ben-Shachar, Yemima Berman, Jaishri Blakeley, Isabel Cordeiro, Ype Elgersma, D Gareth Evans, Michael J Fisher, Ian M Frayling, Joshi George, Susan M Huson, Bronwyn Kerr, Uday Khire, Bruce Korf, Eric Legius, Ludwine Messiaen, Rick van Minkelen, Sheela Nampoothiri, Joanne Ngeow, Luis F Parada, Shubha Phadke, Ashok Pillai, Scott R Plotkin, Ratna Puri, Anup Raji, Vijaya Ramesh, Nancy Ratner, Suma P Shankar, Sheetal Sharda, Anant Tambe, Miikka Vikkula, Brigitte C Widemann, Pierre Wolkenstein, Meena Upadhyaya,

The neurofibromatoses, which include neurofibromatosis type I (NF1), neurofibromatosis type II (NF2), and schwannomatosis, are a group of syndromes characterized by tumor growth in the nervous system. The RASopathies are a group of syndromes caused by germline mutations in genes that encode components of the RAS/mitogen-activated protein kinase (MAPK) pathway. ... Read more >>

Am. J. Med. Genet. A (American journal of medical genetics. Part A)
[2019, 179(6):1091-1097]

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A cerebellopontine angle mouse model for the investigation of tumor biology, hearing, and neurological function in NF2-related vestibular schwannoma.

Jie Chen, Lukas D Landegger, Yao Sun, Jun Ren, Nir Maimon, Limeng Wu, Mei R Ng, John W Chen, Na Zhang, Yingchao Zhao, Xing Gao, Takeshi Fujita, Sylvie Roberge, Peigen Huang, Rakesh K Jain, Scott R Plotkin, Konstantina M Stankovic, Lei Xu,

Neurofibromatosis type II (NF2) is a disease that lacks effective therapies. NF2 is characterized by bilateral vestibular schwannomas (VSs) that cause progressive and debilitating hearing loss, leading to social isolation and increased rates of depression. A major limitation in NF2 basic and translational research is the lack of animal models ... Read more >>

Nat Protoc (Nature protocols)
[2019, 14(2):541-555]

Cited: 1 time

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INNV-20. UTILITY OF TELEHEALTH FOR SPECIALTY NEUROFIBROMATOSIS (NF) CARE.

Justin Jordan, Marlon Seijo, Vanessa Merker, Shivkumar Bhadola, Raquel Thalheimer, Scott R Plotkin,

AbstractBACKGROUNDTelehealth allows for evaluation and management to be delivered over long distances, which may particularly benefit patients with rare neurological diseases.METHODSWe performed a retrospective cohort study of patients receiving telehealth-based care in our NF clinic, and used ArcGIS to calculate driving distance and time from patients home to clinic.RESULTS109 patients ... Read more >>

Neuro-oncology (Neuro-oncology)
[2018, 20(Suppl 6):vi142-vi142]

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Probing tumor microenvironment in patients with newly diagnosed glioblastoma during chemoradiation and adjuvant temozolomide with functional MRI.

K Ina Ly, Bella Vakulenko-Lagun, Kyrre E Emblem, Yangming Ou, Xiao Da, Rebecca A Betensky, Jayashree Kalpathy-Cramer, Dan G Duda, Rakesh K Jain, Andrew S Chi, Scott R Plotkin, Tracy T Batchelor, Gregory Sorensen, Bruce R Rosen, Elizabeth R Gerstner,

Functional MRI may identify critical windows of opportunity for drug delivery and distinguish between early treatment responders and non-responders. Using diffusion-weighted, dynamic contrast-enhanced, and dynamic susceptibility contrast MRI, as well as pro-angiogenic and pro-inflammatory blood markers, we prospectively studied the physiologic tumor-related changes in fourteen newly diagnosed glioblastoma patients during ... Read more >>

Sci Rep (Scientific reports)
[2018, 8(1):17062]

Cited: 1 time

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Mind-Body Treatment for International English-Speaking Adults With Neurofibromatosis via Live Videoconferencing: Protocol for a Single-Blind Randomized Controlled Trial.

Ana-Maria Vranceanu, Emily L Zale, Christopher J Funes, Eric A Macklin, Jessica McCurley, Elyse R Park, Justin T Jordan, Ann Lin, Scott R Plotkin,

BACKGROUND:Neurofibromatoses (NF) are rare genetic conditions associated with substantial psychosocial burden and impaired quality of life (QoL). We developed the first NF-tailored mind-body program (the Relaxation Response Resiliency Program for NF) and adapted it for delivery via live videoconferencing to decrease participation barriers and increase its reach. In a pilot ... Read more >>

JMIR Res Protoc (JMIR research protocols)
[2018, 7(10):e11008]

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Benign Intracranial Tumors.

Justin T Jordan, Scott R Plotkin,

Meningiomas are the most common primary intracranial tumor in adults. With schwannomas, these tumors account for almost all intracranial benign tumors. Despite the benign histologic nature, patients suffer from neurologic symptoms and require treatment. Surgery remains the primary treatment for many such tumors, though radiation may be used either as ... Read more >>

Neurol Clin (Neurologic clinics)
[2018, 36(3):501-516]

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Increasing access to specialty care for rare diseases: a case study using a foundation sponsored clinic network for patients with neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis.

Vanessa L Merker, Annie Dai, Heather B Radtke, Pamela Knight, Justin T Jordan, Scott R Plotkin,

BACKGROUND:Our primary aim was to assess the ability of a non-profit foundation-sponsored clinic network to facilitate access to specialized care for patients with neurofibromatoses (NF), a group of neurogenetic disorders including NF1, NF2, and schwannomatosis (SWN). Our secondary aim was to identify how our findings in NF could be applied ... Read more >>

BMC Health Serv Res (BMC health services research)
[2018, 18(1):668]

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EPH receptor signaling as a novel therapeutic target in NF2-deficient meningioma.

Steven P Angus, Janet L Oblinger, Timothy J Stuhlmiller, Patrick A DeSouza, Roberta L Beauchamp, Luke Witt, Xin Chen, Justin T Jordan, Thomas S K Gilbert, Anat Stemmer-Rachamimov, James F Gusella, Scott R Plotkin, Stephen J Haggarty, Long-Sheng Chang, Gary L Johnson, Vijaya Ramesh, ,

Background:Meningiomas are the most common primary brain tumor in adults, and somatic loss of the neurofibromatosis 2 (NF2) tumor suppressor gene is a frequent genetic event. There is no effective treatment for tumors that recur or continue to grow despite surgery and/or radiation. Therefore, targeted therapies that either delay tumor ... Read more >>

Neuro-oncology (Neuro-oncology)
[2018, 20(9):1185-1196]

Cited: 2 times

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Considerations for development of therapies for cutaneous neurofibroma.

Sharad K Verma, Vincent M Riccardi, Scott R Plotkin, Hubert Weinberg, R Rox Anderson, Jaishri O Blakeley, Kurt Jarnagin, James Lee,

OBJECTIVE:The only therapies currently available for cutaneous neurofibromas (cNF) are procedural. The goals of the Therapies Development Working Group were to (1) summarize currently available treatment options for cNF, (2) define key considerations for drug discovery and development generally, and specifically for cNF, and (3) outline recommendations for the successful ... Read more >>

Neurology (Neurology)
[2018, 91(2 Suppl 1):S21-S30]

Cited: 2 times

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Cutaneous neurofibromas: Current clinical and pathologic issues.

Nicolas Ortonne, Pierre Wolkenstein, Jaishri O Blakeley, Bruce Korf, Scott R Plotkin, Vincent M Riccardi, Douglas C Miller, Susan Huson, Juha Peltonen, Andrew Rosenberg, Steven L Carroll, Sharad K Verma, Victor Mautner, Meena Upadhyaya, Anat Stemmer-Rachamimov,

OBJECTIVE:To present the current terminology and natural history of neurofibromatosis 1 (NF1) cutaneous neurofibromas (cNF). METHODS:NF1 experts from various research and clinical backgrounds reviewed the terms currently in use for cNF as well as the clinical, histologic, and radiographic features of these tumors using published and unpublished data. RESULTS:Neurofibromas develop ... Read more >>

Neurology (Neurology)
[2018, 91(2 Suppl 1):S5-S13]

Cited: 3 times

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Traditional and systems biology based drug discovery for the rare tumor syndrome neurofibromatosis type 2.

, Robert Allaway, Steve P Angus, Roberta L Beauchamp, Jaishri O Blakeley, Marga Bott, Sarah S Burns, Annemarie Carlstedt, Long-Sheng Chang, Xin Chen, D Wade Clapp, Patrick A Desouza, Serkan Erdin, Cristina Fernandez-Valle, Justin Guinney, James F Gusella, Stephen J Haggarty, Gary L Johnson, Salvatore La Rosa, Helen Morrison, Alejandra M Petrilli, Scott R Plotkin, Abhishek Pratap, Vijaya Ramesh, Noah Sciaky, Anat Stemmer-Rachamimov, Tim J Stuhlmiller, Michael E Talkowski, D Bradley Welling, Charles W Yates, Jon S Zawistowski, Wen-Ning Zhao,

Neurofibromatosis 2 (NF2) is a rare tumor suppressor syndrome that manifests with multiple schwannomas and meningiomas. There are no effective drug therapies for these benign tumors and conventional therapies have limited efficacy. Various model systems have been created and several drug targets have been implicated in NF2-driven tumorigenesis based on ... Read more >>

PLoS ONE (PloS one)
[2018, 13(6):e0197350]

Cited: 1 time

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Improvement in Patient-reported Hearing After Treatment With Bevacizumab in People With Neurofibromatosis Type 2.

Victoria Huang, Amanda L Bergner, Chris Halpin, Vanessa L Merker, Monica R Sheridan, Brigitte C Widemann, Jaishri O Blakeley, Scott R Plotkin,

OBJECTIVE:Assess patient-reported outcomes (PRO) for hearing and tinnitus relative to clinical hearing assessment in people with neurofibromatosis 2 (NF2) associated hearing loss. STUDY DESIGN:Prospective, open label, phase-II clinical trial with PRO administered pre-, post-, and after treatment. SETTING:Three tertiary referral centers. PATIENTS:Fourteen patients with NF2, median age of 30 years ... Read more >>

Otol. Neurotol. (Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology)
[2018, 39(5):632-638]

Cited: 2 times

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Neurofibromatosis and Schwannomatosis.

Scott R Plotkin, Antje Wick,

Neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis are a group of related classically inherited but often times sporadic tumor suppressor syndromes. Neuro-oncologists should recognize these syndromes, initiate necessary tests in patients with a clinical suspicion, and support genetic counseling of patients and families. In this review, clinical presentation, diagnostic criteria, day-to-day ... Read more >>

Semin Neurol (Seminars in neurology)
[2018, 38(1):73-85]

Cited: 3 times

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Targeting the cMET pathway augments radiation response without adverse effect on hearing in NF2 schwannoma models.

Yingchao Zhao, Pinan Liu, Na Zhang, Jie Chen, Lukas D Landegger, Limeng Wu, Fu Zhao, Yanxia Zhao, Yanling Zhang, Jing Zhang, Takeshi Fujita, Anat Stemmer-Rachamimov, Gino B Ferraro, Hao Liu, Alona Muzikansky, Scott R Plotkin, Konstantina M Stankovic, Rakesh K Jain, Lei Xu,

Neurofibromatosis type II (NF2) is a disease that needs new solutions. Vestibular schwannoma (VS) growth causes progressive hearing loss, and the standard treatment, including surgery and radiotherapy, can further damage the nerve. There is an urgent need to identify an adjunct therapy that, by enhancing the efficacy of radiation, can ... Read more >>

Proc. Natl. Acad. Sci. U.S.A. (Proceedings of the National Academy of Sciences of the United States of America)
[2018, 115(9):E2077-E2084]

Cited: 1 time

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Pain correlates with germline mutation in schwannomatosis.

Justin T Jordan, Miriam J Smith, James A Walker, Serkan Erdin, Michael E Talkowski, Vanessa L Merker, Vijaya Ramesh, Wenli Cai, Gordon J Harris, Miriam A Bredella, Marlon Seijo, Alessandra Suuberg, James F Gusella, Scott R Plotkin,

Schwannomatosis has been linked to germline mutations in the SMARCB1 and LZTR1 genes, and is frequently associated with pain.In a cohort study, we assessed the mutation status of 37 patients with clinically diagnosed schwannomatosis and compared to clinical data, whole body MRI (WBMRI), visual analog pain scale, and Short Form ... Read more >>

Medicine (Baltimore) (Medicine)
[2018, 97(5):e9717]

Cited: 1 time

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Genotype-Phenotype Correlation in NF1: Evidence for a More Severe Phenotype Associated with Missense Mutations Affecting NF1 Codons 844-848.

Magdalena Koczkowska, Yunjia Chen, Tom Callens, Alicia Gomes, Angela Sharp, Sherrell Johnson, Meng-Chang Hsiao, Zhenbin Chen, Meena Balasubramanian, Christopher P Barnett, Troy A Becker, Shay Ben-Shachar, Debora R Bertola, Jaishri O Blakeley, Emma M M Burkitt-Wright, Alison Callaway, Melissa Crenshaw, Karin S Cunha, Mitch Cunningham, Maria D D'Agostino, Karin Dahan, Alessandro De Luca, Anne Destrée, Radhika Dhamija, Marica Eoli, D Gareth R Evans, Patricia Galvin-Parton, Jaya K George-Abraham, Karen W Gripp, Jose Guevara-Campos, Neil A Hanchard, Concepcion Hernández-Chico, LaDonna Immken, Sandra Janssens, Kristi J Jones, Beth A Keena, Aaina Kochhar, Jan Liebelt, Arelis Martir-Negron, Maurice J Mahoney, Isabelle Maystadt, Carey McDougall, Meriel McEntagart, Nancy Mendelsohn, David T Miller, Geert Mortier, Jenny Morton, John Pappas, Scott R Plotkin, Dinel Pond, Kenneth Rosenbaum, Karol Rubin, Laura Russell, Lane S Rutledge, Veronica Saletti, Rhonda Schonberg, Allison Schreiber, Meredith Seidel, Elizabeth Siqveland, David W Stockton, Eva Trevisson, Nicole J Ullrich, Meena Upadhyaya, Rick van Minkelen, Helene Verhelst, Margaret R Wallace, Yoon-Sim Yap, Elaine Zackai, Jonathan Zonana, Vickie Zurcher, Kathleen Claes, Yolanda Martin, Bruce R Korf, Eric Legius, Ludwine M Messiaen,

Neurofibromatosis type 1 (NF1), a common genetic disorder with a birth incidence of 1:2,000-3,000, is characterized by a highly variable clinical presentation. To date, only two clinically relevant intragenic genotype-phenotype correlations have been reported for NF1 missense mutations affecting p.Arg1809 and a single amino acid deletion p.Met922del. Both variants predispose ... Read more >>

Am. J. Hum. Genet. (American journal of human genetics)
[2018, 102(1):69-87]

Cited: 18 times

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Health literacy assessment in adults with neurofibromatosis: electronic and short-form measurement using FCCHL and Health LiTT.

Vanessa L Merker, Sarah McDannold, Eric Riklin, Mojtaba Talaei-Khoei, Monica R Sheridan, Justin T Jordan, Scott R Plotkin, Ana-Maria Vranceanu,

Determining health literacy level is an important prerequisite for effective patient education. We assessed multiple dimensions of health literacy and sociodemographic predictors of health literacy in patients with neurofibromatosis. In 86 individuals with a confirmed diagnosis of neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), or schwannomatosis, we assessed health literacy status ... Read more >>

J. Neurooncol. (Journal of neuro-oncology)
[2018, 136(2):335-342]

Cited: 0 times

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Volumetric MRI Analysis of Plexiform Neurofibromas in Neurofibromatosis Type 1: Comparison of Two Methods.

Wenli Cai, Seth M Steinberg, Miriam A Bredella, Gina Basinsky, Bhanusupriya Somarouthu, Scott R Plotkin, Jeffrey Solomon, Brigitte C Widemann, Gordon J Harris, Eva Dombi,

OBJECTIVES:Plexiform neurofibromas (PNs) are complex, histologically benign peripheral nerve sheath tumors that are challenging to measure by simple line measurements. Computer-aided volumetric segmentation of PN has become the recommended method to assess response in clinical trials directed at PN. Different methods for volumetric analysis of PN have been developed. The ... Read more >>

Acad Radiol (Academic radiology)
[2018, 25(2):144-152]

Cited: 0 times

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Anti-VEGF treatment improves neurological function in tumors of the nervous system.

Na Zhang, Jie Chen, Gino B Ferraro, Limeng Wu, Meenal Datta, Rakesh K Jain, Scott R Plotkin, Anat Stemmer-Rachamimov, Lei Xu,

Research of various diseases of the nervous system has shown that VEGF has direct neuroprotective effects in the central and peripheral nervous systems, and indirect effects on improving neuronal vessel perfusion which leads to nerve protection. In the tumors of the nervous system, VEGF plays a critical role in tumor ... Read more >>

Exp. Neurol. (Experimental neurology)
[2018, 299(Pt B):326-333]

Cited: 6 times

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