Current whole-body MRI applications in the neurofibromatoses: NF1, NF2, and schwannomatosis

Shivani Ahlawat, Laura M Fayad, Muhammad Shayan Khan, Miriam A Bredella, Gordon J Harris, D Gareth Evans, Said Farschtschi, Michael A Jacobs, Avneesh Chhabra, Johannes M Salamon, Ralph Wenzel, Victor F Mautner, Eva Dombi, Wenli Cai, Scott R Plotkin, Jaishri O Blakeley, Whole Body MRI Committee for the REiNS International Collaboration, REiNS International Collaboration Members 2016, Shivani Ahlawat, Srivandana Akshintala, Jeffrey Allen, Simone Ardern-Holmes, Robert Avery, Amedeo Azizi, Dusica Babovic-Vuksanovic, Annette Bakker, Andrea Baldwin, Fred Barker, Amanda Bergner, Chetan Bettegowda, Sucharita Bhaumik, Larissa Bilaniuk, Kim Bischoff, Jaishri Blakely, Diana Bradford, Miriam Bredella, Wenli Cai, John Carino, Avneesh Chabra, Patricia Ciavarelli, Wade Clapp, Stephen Connor, Albert Cornelius, Tambra Dahlheimer, Stephanie Davis, Peter de Blank, Vidya Dhote, Joni Doherty, Eva Dombi, William Dudley, Rachel Ershler, D Gareth Evans, Laura Fayad, Cristina Fernandez-Valle, Rosalie Ferner, Michael Fisher, Barbara Franklin, Giulia Fulci, Tracy Galloway, Kathy Gardner, Richard Gedrich, Marco Giovannini, Amy Goldstein, Anne Goodwin, Stephane Goutagny, David Gutmann, Theresa Hadlock, Chris Halpin, C Oliver Hanemann, Kristina Hardy, Gordon Harris, Desiree Headley, Gena Heidary, Jonathan Heller, Cynthia Hingtgen, Trent Hummel, Susan Huson, Michael Jacobs, Jennifer Janusz, Diego Jaramillo, Justin Jordan, Allen Julian, Michel Kalamarides, Matthias Karajannis, Bonnie Klein-Tasman, Pamela Knight, Bruce Korf, Shannon Langmead, Theresa LaVallee, Fawn Leigh, Donita Lightner, Carol Lin, Robert Listernick, Grant Liu, Mevo Marco, Carole Marcus, Gabriella Mariani, Staci Martin, Victor Mautner, Vanessa Merker, Michael Ferguson, Chris Moertel, Jill Morris, Katrina Morris, Kathryn North, Fabio Nunes, Roger Packer, Laura Papi, Allyson Parry, Neha Patel, Jonathan Payne, Karen Peluso, Sebastian Perreault, Scott Plotkin, Tina Poussaint, Nancy Ratner, Karlyne Reilly, Vincent Riccardi, Kent Robertson, Claas Rohl, Deborah Rukin Gold, La Rosa Salvatore, Laura Schaffner Gray, Elizabeth Schorry, Claire Semerjian, Monica Sheridan, Chie-Schin Shih, Carolyn Sidor, William Slattery 3rd, Miriam Smith, Kathy Sommer, Marigo Stathis, Matt Steensma, Anat Stemmer-Rachamimov, David Stevenson, Kari Struemph, Lara Sullivan, Mary Anne Tamula, Mary Thomas, Heather Thompson, James Tonsgard, Nicole Ullrich, Sharad Verma, David Viskochil, Ana-Maria Vranceanu, Ute Wahllander, Karin Walsh, Bradley Welling, Ralph Wenzel, Trish Whitcomb, Brigitte Widemann, Victoria Williams, David Wolf, Pamela Wolters, Kaleb Yohay, Shivani Ahlawat, Laura M Fayad, Muhammad Shayan Khan, Miriam A Bredella, Gordon J Harris, D Gareth Evans, Said Farschtschi, Michael A Jacobs, Avneesh Chhabra, Johannes M Salamon, Ralph Wenzel, Victor F Mautner, Eva Dombi, Wenli Cai, Scott R Plotkin, Jaishri O Blakeley, Whole Body MRI Committee for the REiNS International Collaboration, REiNS International Collaboration Members 2016, Shivani Ahlawat, Srivandana Akshintala, Jeffrey Allen, Simone Ardern-Holmes, Robert Avery, Amedeo Azizi, Dusica Babovic-Vuksanovic, Annette Bakker, Andrea Baldwin, Fred Barker, Amanda Bergner, Chetan Bettegowda, Sucharita Bhaumik, Larissa Bilaniuk, Kim Bischoff, Jaishri Blakely, Diana Bradford, Miriam Bredella, Wenli Cai, John Carino, Avneesh Chabra, Patricia Ciavarelli, Wade Clapp, Stephen Connor, Albert Cornelius, Tambra Dahlheimer, Stephanie Davis, Peter de Blank, Vidya Dhote, Joni Doherty, Eva Dombi, William Dudley, Rachel Ershler, D Gareth Evans, Laura Fayad, Cristina Fernandez-Valle, Rosalie Ferner, Michael Fisher, Barbara Franklin, Giulia Fulci, Tracy Galloway, Kathy Gardner, Richard Gedrich, Marco Giovannini, Amy Goldstein, Anne Goodwin, Stephane Goutagny, David Gutmann, Theresa Hadlock, Chris Halpin, C Oliver Hanemann, Kristina Hardy, Gordon Harris, Desiree Headley, Gena Heidary, Jonathan Heller, Cynthia Hingtgen, Trent Hummel, Susan Huson, Michael Jacobs, Jennifer Janusz, Diego Jaramillo, Justin Jordan, Allen Julian, Michel Kalamarides, Matthias Karajannis, Bonnie Klein-Tasman, Pamela Knight, Bruce Korf, Shannon Langmead, Theresa LaVallee, Fawn Leigh, Donita Lightner, Carol Lin, Robert Listernick, Grant Liu, Mevo Marco, Carole Marcus, Gabriella Mariani, Staci Martin, Victor Mautner, Vanessa Merker, Michael Ferguson, Chris Moertel, Jill Morris, Katrina Morris, Kathryn North, Fabio Nunes, Roger Packer, Laura Papi, Allyson Parry, Neha Patel, Jonathan Payne, Karen Peluso, Sebastian Perreault, Scott Plotkin, Tina Poussaint, Nancy Ratner, Karlyne Reilly, Vincent Riccardi, Kent Robertson, Claas Rohl, Deborah Rukin Gold, La Rosa Salvatore, Laura Schaffner Gray, Elizabeth Schorry, Claire Semerjian, Monica Sheridan, Chie-Schin Shih, Carolyn Sidor, William Slattery 3rd, Miriam Smith, Kathy Sommer, Marigo Stathis, Matt Steensma, Anat Stemmer-Rachamimov, David Stevenson, Kari Struemph, Lara Sullivan, Mary Anne Tamula, Mary Thomas, Heather Thompson, James Tonsgard, Nicole Ullrich, Sharad Verma, David Viskochil, Ana-Maria Vranceanu, Ute Wahllander, Karin Walsh, Bradley Welling, Ralph Wenzel, Trish Whitcomb, Brigitte Widemann, Victoria Williams, David Wolf, Pamela Wolters, Kaleb Yohay

Abstract

Objectives: The Response Evaluation in Neurofibromatosis and Schwannomatosis (REiNS) International Collaboration Whole-Body MRI (WB-MRI) Working Group reviewed the existing literature on WB-MRI, an emerging technology for assessing disease in patients with neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN), to recommend optimal image acquisition and analysis methods to enable WB-MRI as an endpoint in NF clinical trials.

Methods: A systematic process was used to review all published data about WB-MRI in NF syndromes to assess diagnostic accuracy, feasibility and reproducibility, and data about specific techniques for assessment of tumor burden, characterization of neoplasms, and response to therapy.

Results: WB-MRI at 1.5T or 3.0T is feasible for image acquisition. Short tau inversion recovery (STIR) sequence is used in all investigations to date, suggesting consensus about the utility of this sequence for detection of WB tumor burden in people with NF. There are insufficient data to support a consensus statement about the optimal imaging planes (axial vs coronal) or 2D vs 3D approaches. Functional imaging, although used in some NF studies, has not been systematically applied or evaluated. There are no comparative studies between regional vs WB-MRI or evaluations of WB-MRI reproducibility.

Conclusions: WB-MRI is feasible for identifying tumors using both 1.5T and 3.0T systems. The STIR sequence is a core sequence. Additional investigation is needed to define the optimal approach for volumetric analysis, the reproducibility of WB-MRI in NF, and the diagnostic performance of WB-MRI vs regional MRI.

© 2016 American Academy of Neurology.

Figures

Figure. Whole-body MRI in neurofibromatosis
Figure. Whole-body MRI in neurofibromatosis
(A) A person with schwannomatosis. (B) A person with neurofibromatosis type 1. In both images, the range of field of view is apparent depending on the table length and the size of the person being imaged and in both images several tumors distributed across anatomic regions can be identified. In (B), some of the artifact that can occur affecting interpretation is apparent.

Source: PubMed

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