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{"title"=>"Quantitative assessment of whole-body tumor burden in adult patients with neurofibromatosis", "type"=>"journal", "authors"=>[{"first_name"=>"Scott R.", "last_name"=>"Plotkin", "scopus_author_id"=>"7203070176"}, {"first_name"=>"Miriam A.", "last_name"=>"Bredella", "scopus_author_id"=>"6602236666"}, {"first_name"=>"Wenli", "last_name"=>"Cai", "scopus_author_id"=>"37461112100"}, {"first_name"=>"Ara", "last_name"=>"Kassarjian", "scopus_author_id"=>"6601911000"}, {"first_name"=>"Gordon J.", "last_name"=>"Harris", "scopus_author_id"=>"7403158766"}, {"first_name"=>"Sonia", "last_name"=>"Esparza", "scopus_author_id"=>"55203129800"}, {"first_name"=>"Vanessa L.", "last_name"=>"Merker", "scopus_author_id"=>"54416172800"}, {"first_name"=>"Lance L.", "last_name"=>"Munn", "scopus_author_id"=>"7006040544"}, {"first_name"=>"Alona", "last_name"=>"Muzikansky", "scopus_author_id"=>"6602976834"}, {"first_name"=>"Manor", "last_name"=>"Askenazi", "scopus_author_id"=>"35344685600"}, {"first_name"=>"Rosa", "last_name"=>"Nguyen", "scopus_author_id"=>"35330466900"}, {"first_name"=>"Ralph", "last_name"=>"Wenzel", "scopus_author_id"=>"19739851500"}, {"first_name"=>"Victor F.", "last_name"=>"Mautner", "scopus_author_id"=>"7005686458"}], "year"=>2012, "source"=>"PLoS ONE", "identifiers"=>{"scopus"=>"2-s2.0-84860459532", "sgr"=>"84860459532", "issn"=>"19326203", "doi"=>"10.1371/journal.pone.0035711", "pmid"=>"22558206", "isbn"=>"1932-6203", "pui"=>"364720821"}, "id"=>"2a6bc65e-08c6-37e8-a30f-5d1d3ebaa480", "abstract"=>"PURPOSE: Patients with neurofibromatosis 1 (NF1), NF2, and schwannomatosis are at risk for multiple nerve sheath tumors and premature mortality. Traditional magnetic resonance imaging (MRI) has limited ability to assess disease burden accurately. The aim of this study was to establish an international cohort of patients with quantified whole-body internal tumor burden and to correlate tumor burden with clinical features of disease.METHODS: We determined the number, volume, and distribution of internal nerve sheath tumors in patients using whole-body MRI (WBMRI) and three-dimensional computerized volumetry. We quantified the distribution of tumor volume across body regions and used unsupervised cluster analysis to group patients based on tumor distribution. We correlated the presence and volume of internal tumors with disease-related and demographic factors.RESULTS: WBMRI identified 1286 tumors in 145/247 patients (59%). Schwannomatosis patients had the highest prevalence of tumors (P = 0.03), but NF1 patients had the highest median tumor volume (P = 0.02). Tumor volume was unevenly distributed across body regions with overrepresentation of the head/neck and pelvis. Risk factors for internal nerve sheath tumors included decreasing numbers of café-au-lait macules in NF1 patients (P = 0.003) and history of skeletal abnormalities in NF2 patients (P = 0.09). Risk factors for higher tumor volume included female gender (P = 0.05) and increasing subcutaneous neurofibromas (P = 0.03) in NF1 patients, absence of cutaneous schwannomas in NF2 patients (P = 0.06), and increasing age in schwannomatosis patients (p = 0.10).CONCLUSION: WBMRI provides a comprehensive phenotype of neurofibromatosis patients, identifies distinct anatomic subgroups, and provides the basis for investigating molecular biomarkers that correlate with unique disease manifestations", "link"=>"http://www.mendeley.com/research/quantitative-assessment-wholebody-tumor-burden-adult-patients-neurofibromatosis", "reader_count"=>32, "reader_count_by_academic_status"=>{"Unspecified"=>2, "Professor > Associate Professor"=>3, "Researcher"=>8, "Student > Doctoral Student"=>2, "Student > Ph. D. Student"=>2, "Student > Postgraduate"=>5, "Other"=>4, "Student > Master"=>3, "Student > Bachelor"=>1, "Lecturer > Senior Lecturer"=>1, "Professor"=>1}, "reader_count_by_user_role"=>{"Unspecified"=>2, "Professor > Associate Professor"=>3, "Researcher"=>8, "Student > Doctoral Student"=>2, "Student > Ph. D. Student"=>2, "Student > Postgraduate"=>5, "Other"=>4, "Student > Master"=>3, "Student > Bachelor"=>1, "Lecturer > Senior Lecturer"=>1, "Professor"=>1}, "reader_count_by_subject_area"=>{"Unspecified"=>6, "Biochemistry, Genetics and Molecular Biology"=>3, "Medicine and Dentistry"=>14, "Agricultural and Biological Sciences"=>3, "Neuroscience"=>2, "Pharmacology, Toxicology and Pharmaceutical Science"=>1, "Social Sciences"=>1, "Computer Science"=>2}, "reader_count_by_subdiscipline"=>{"Medicine and Dentistry"=>{"Medicine and Dentistry"=>14}, "Neuroscience"=>{"Neuroscience"=>2}, "Social Sciences"=>{"Social Sciences"=>1}, "Agricultural and Biological Sciences"=>{"Agricultural and Biological Sciences"=>3}, "Computer Science"=>{"Computer Science"=>2}, "Biochemistry, Genetics and Molecular Biology"=>{"Biochemistry, Genetics and Molecular Biology"=>3}, "Unspecified"=>{"Unspecified"=>6}, "Pharmacology, Toxicology and Pharmaceutical Science"=>{"Pharmacology, Toxicology and Pharmaceutical Science"=>1}}, "group_count"=>2}

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• http://doi.org/10.1007/s00256-016-2522-4
• http://doi.org/10.1172/jci.insight.98601
• http://doi.org/10.1002/ajmg.a.35760
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• {"id"=>"331528405445124097", "text"=>"Cool interactive figure \nhttp://t.co/RetUBu3GrI shows whole-body Tumor Burden of Adult Neurofibromatosis http://t.co/nNUJTHkiUy #ICBP", "created_at"=>"2013-05-06T21:58:26Z", "user"=>"ara_anderson", "user_name"=>"Sandy Anderson", "user_profile_image"=>"http://a0.twimg.com/profile_images/280195912/sandy_icon2_normal.png"}

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Figshare

• {"files"=>["https://ndownloader.figshare.com/files/645843"], "description"=>"<p>Odds ratio for the presence of internal nerve sheath tumors in NF1, NF2, and schwannomatosis patients, according to clinical and demographic characteristics. Odds ratios and 95% confidence intervals were calculated with the use of logistic regression analsysis. Squares indicate odds ratios and horizontal lines indicate 95% confidence intervals. P values are for the odds ratios.</p>", "links"=>[], "tags"=>["ratios", "sheath"], "article_id"=>316320, "categories"=>["Cancer", "Genetics", "Cell Biology", "Neuroscience"], "users"=>["Scott R. Plotkin", "Miriam A. Bredella", "Wenli Cai", "Ara Kassarjian", "Gordon J. Harris", "Sonia Esparza", "Vanessa L. Merker", "Lance L. Munn", "Alona Muzikansky", "Manor Askenazi", "Rosa Nguyen", "Ralph Wenzel", "Victor F. Mautner"], "doi"=>"https://dx.doi.org/10.1371/journal.pone.0035711.g003", "stats"=>{"downloads"=>1, "page_views"=>10, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Odds_ratios_for_the_presence_of_internal_nerve_sheath_tumors_/316320", "title"=>"Odds ratios for the presence of internal nerve sheath tumors.", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-20 05:49:46"}
• {"files"=>["https://ndownloader.figshare.com/files/646194"], "description"=>"<p>Whole-body tumor number and volume in patients with at least 1 internal tumor.</p>", "links"=>[], "tags"=>["patients"], "article_id"=>316672, "categories"=>["Cancer", "Genetics", "Cell Biology", "Neuroscience"], "users"=>["Scott R. Plotkin", "Miriam A. Bredella", "Wenli Cai", "Ara Kassarjian", "Gordon J. Harris", "Sonia Esparza", "Vanessa L. Merker", "Lance L. Munn", "Alona Muzikansky", "Manor Askenazi", "Rosa Nguyen", "Ralph Wenzel", "Victor F. Mautner"], "doi"=>"https://dx.doi.org/10.1371/journal.pone.0035711.t003", "stats"=>{"downloads"=>0, "page_views"=>15, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Whole_body_tumor_number_and_volume_in_patients_with_at_least_1_internal_tumor_/316672", "title"=>"Whole-body tumor number and volume in patients with at least 1 internal tumor.", "pos_in_sequence"=>0, "defined_type"=>3, "published_date"=>"2013-02-20 05:51:32"}
• {"files"=>["https://ndownloader.figshare.com/files/645608"], "description"=>"<p>Tumor type was defined according to the radiologic appearance without need for pathological diagnosis. Tumors that were locally circumscribed on MRI scan were classified as circumscribed tumors and those that were invasive or involved multiple nerves were classified as plexiform tumors.</p>", "links"=>[], "tags"=>["sheath", "tumors", "whole-body"], "article_id"=>316069, "categories"=>["Cancer", "Genetics", "Cell Biology", "Neuroscience"], "users"=>["Scott R. Plotkin", "Miriam A. Bredella", "Wenli Cai", "Ara Kassarjian", "Gordon J. Harris", "Sonia Esparza", "Vanessa L. Merker", "Lance L. Munn", "Alona Muzikansky", "Manor Askenazi", "Rosa Nguyen", "Ralph Wenzel", "Victor F. Mautner"], "doi"=>"https://dx.doi.org/10.1371/journal.pone.0035711.g001", "stats"=>{"downloads"=>1, "page_views"=>20, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Appearance_of_internal_nerve_sheath_tumors_on_Whole_Body_MRI_/316069", "title"=>"Appearance of internal nerve sheath tumors on Whole-Body MRI.", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-20 05:48:31"}
• {"files"=>["https://ndownloader.figshare.com/files/645724"], "description"=>"<p>Waterfall plot of tumor count (panel A) and tumor volume (Panel B) in 145 patients with at least one internal tumor. Anatomic distribution of relative tumor volume given as a percentage of whole-body volume (Panel C) and corrected for volume per body part (Panel D).</p>", "links"=>[], "tags"=>["anatomic", "sheath"], "article_id"=>316198, "categories"=>["Cancer", "Genetics", "Cell Biology", "Neuroscience"], "users"=>["Scott R. Plotkin", "Miriam A. Bredella", "Wenli Cai", "Ara Kassarjian", "Gordon J. Harris", "Sonia Esparza", "Vanessa L. Merker", "Lance L. Munn", "Alona Muzikansky", "Manor Askenazi", "Rosa Nguyen", "Ralph Wenzel", "Victor F. Mautner"], "doi"=>"https://dx.doi.org/10.1371/journal.pone.0035711.g002", "stats"=>{"downloads"=>0, "page_views"=>11, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Number_volume_and_anatomic_distribution_of_internal_nerve_sheath_tumors_/316198", "title"=>"Number, volume, and anatomic distribution of internal nerve sheath tumors.", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-20 05:49:10"}
• {"files"=>["https://ndownloader.figshare.com/files/646300"], "description"=>"<p>Demographic features of the 247 patients who underwent whole-body MRI.</p>", "links"=>[], "tags"=>["247", "patients", "underwent", "whole-body"], "article_id"=>316776, "categories"=>["Cancer", "Genetics", "Cell Biology", "Neuroscience"], "users"=>["Scott R. Plotkin", "Miriam A. Bredella", "Wenli Cai", "Ara Kassarjian", "Gordon J. Harris", "Sonia Esparza", "Vanessa L. Merker", "Lance L. Munn", "Alona Muzikansky", "Manor Askenazi", "Rosa Nguyen", "Ralph Wenzel", "Victor F. Mautner"], "doi"=>"https://dx.doi.org/10.1371/journal.pone.0035711.t001", "stats"=>{"downloads"=>4, "page_views"=>9, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Demographic_features_of_the_247_patients_who_underwent_whole_body_MRI_/316776", "title"=>"Demographic features of the 247 patients who underwent whole-body MRI.", "pos_in_sequence"=>0, "defined_type"=>3, "published_date"=>"2013-02-20 05:52:01"}
• {"files"=>["https://ndownloader.figshare.com/files/646242"], "description"=>"<p>Clinical features of the 247 patients who underwent whole-body MRI.</p>", "links"=>[], "tags"=>["247", "patients", "underwent", "whole-body"], "article_id"=>316717, "categories"=>["Cancer", "Genetics", "Cell Biology", "Neuroscience"], "users"=>["Scott R. Plotkin", "Miriam A. Bredella", "Wenli Cai", "Ara Kassarjian", "Gordon J. Harris", "Sonia Esparza", "Vanessa L. Merker", "Lance L. Munn", "Alona Muzikansky", "Manor Askenazi", "Rosa Nguyen", "Ralph Wenzel", "Victor F. Mautner"], "doi"=>"https://dx.doi.org/10.1371/journal.pone.0035711.t002", "stats"=>{"downloads"=>0, "page_views"=>7, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Clinical_features_of_the_247_patients_who_underwent_whole_body_MRI_/316717", "title"=>"Clinical features of the 247 patients who underwent whole-body MRI.", "pos_in_sequence"=>0, "defined_type"=>3, "published_date"=>"2013-02-20 05:51:46"}
• {"files"=>["https://ndownloader.figshare.com/files/646111"], "description"=>"<p>Patients older than 40 are represented in red; patients 40 or younger are shown in green. These two patient subpopulations differed significantly in relative tumor burden along the craniocaudal axis (p = 0·003 by permutation test) with patients 40 or younger having a higher percentage of tumor volume in the pelvis and legs than patients over 40.</p>", "links"=>[], "tags"=>["patients", "younger", "older"], "article_id"=>316590, "categories"=>["Cancer", "Genetics", "Cell Biology", "Neuroscience"], "users"=>["Scott R. Plotkin", "Miriam A. Bredella", "Wenli Cai", "Ara Kassarjian", "Gordon J. Harris", "Sonia Esparza", "Vanessa L. Merker", "Lance L. Munn", "Alona Muzikansky", "Manor Askenazi", "Rosa Nguyen", "Ralph Wenzel", "Victor F. Mautner"], "doi"=>"https://dx.doi.org/10.1371/journal.pone.0035711.g005", "stats"=>{"downloads"=>0, "page_views"=>1, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Relative_tumor_burden_of_patients_younger_and_older_than_40_/316590", "title"=>"Relative tumor burden of patients younger and older than 40.", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-20 05:51:06"}
• {"files"=>["https://ndownloader.figshare.com/files/645999"], "description"=>"<p>(This figure is an interactive display. It is currently available for at <a href=\"http://www.wholebodymri.org\" target=\"_blank\">www.wholebodymri.org</a>). Unsupervised hierarchical clustering of relative tumor volume across body region (head/neck, trunk, extremities) for 145 subjects with internal nerve sheath tumors. Total tumor volumes were combined and scaled for each patient. Regions with higher tumor burden are shown in yellow. Individual patients are represented as columns and the maximum intensity projection (MIP) of the whole-body MRI scan is shown to the right of the clustering figure. For each patient, the Gini coefficient is shown in the lower left and the whole-body tumor volume in the lower middle.</p>", "links"=>[], "tags"=>["unsupervised", "hierarchical", "clustering", "patients"], "article_id"=>316476, "categories"=>["Cancer", "Genetics", "Cell Biology", "Neuroscience"], "users"=>["Scott R. Plotkin", "Miriam A. Bredella", "Wenli Cai", "Ara Kassarjian", "Gordon J. Harris", "Sonia Esparza", "Vanessa L. Merker", "Lance L. Munn", "Alona Muzikansky", "Manor Askenazi", "Rosa Nguyen", "Ralph Wenzel", "Victor F. Mautner"], "doi"=>"https://dx.doi.org/10.1371/journal.pone.0035711.g004", "stats"=>{"downloads"=>4, "page_views"=>7, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Results_of_unsupervised_hierarchical_clustering_of_patients_based_on_relative_tumor_volume_across_body_regions_/316476", "title"=>"Results of unsupervised hierarchical clustering of patients based on relative tumor volume across body regions.", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-20 05:50:32"}

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Relative Metric

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F1000Prime