Shivani Ahlawat1, Laura M Fayad2, Muhammad Shayan Khan2, Miriam A Bredella2, Gordon J Harris2, D Gareth Evans2, Said Farschtschi2, Michael A Jacobs2, Avneesh Chhabra2, Johannes M Salamon2, Ralph Wenzel2, Victor F Mautner2, Eva Dombi2, Wenli Cai2, Scott R Plotkin2, Jaishri O Blakeley2. 1. From The Russell H. Morgan Department of Radiology and Radiological Science (S.A., L.M.F., M.A.J.), Sidney Kimmel Comprehensive Cancer Center (M.A.J.), and Department of Neurology (J.O.B.), Johns Hopkins University, Baltimore, MD; Khyber Medical College (M.S.K.), Peshawar, Pakistan; Department of Radiology (M.A.B., G.J.H., W.C.), Massachusetts General Hospital and Harvard Medical School, Boston; Genomic Medicine (D.G.E.), Manchester Academic Health Science Centre, The University of Manchester, UK; Department of Neurology (S.F., V.F.M.), University Medical Center Hamburg-Eppendorf, Hamburg, Germany; Radiology & Orthopedic Surgery (A.C.), UT Southwestern Medical Center, Dallas, TX; Department of Diagnostic and Interventional Radiology (J.M.S.), University Hospital Hamburg-Eppendorf; Radiological Practice Altona (R.W.), Hamburg, Germany; Pediatric Oncology Branch (E.D.), National Cancer Institute, Bethesda, MD; and Department of Neurology and Cancer Center (S.R.P.), Massachusetts General Hospital, Boston. sahlawa1@jhmi.edu. 2. From The Russell H. Morgan Department of Radiology and Radiological Science (S.A., L.M.F., M.A.J.), Sidney Kimmel Comprehensive Cancer Center (M.A.J.), and Department of Neurology (J.O.B.), Johns Hopkins University, Baltimore, MD; Khyber Medical College (M.S.K.), Peshawar, Pakistan; Department of Radiology (M.A.B., G.J.H., W.C.), Massachusetts General Hospital and Harvard Medical School, Boston; Genomic Medicine (D.G.E.), Manchester Academic Health Science Centre, The University of Manchester, UK; Department of Neurology (S.F., V.F.M.), University Medical Center Hamburg-Eppendorf, Hamburg, Germany; Radiology & Orthopedic Surgery (A.C.), UT Southwestern Medical Center, Dallas, TX; Department of Diagnostic and Interventional Radiology (J.M.S.), University Hospital Hamburg-Eppendorf; Radiological Practice Altona (R.W.), Hamburg, Germany; Pediatric Oncology Branch (E.D.), National Cancer Institute, Bethesda, MD; and Department of Neurology and Cancer Center (S.R.P.), Massachusetts General Hospital, Boston.
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.
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.
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