Imaging Evaluation of Plexiform Neurofibromas in Neurofibromatosis Type 1: A Survey-Based Assessment
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Imaging Evaluation of Plexiform Neurofibromas in Neurofibromatosis Type 1: A Survey-Based Assessment. / Ahlawat, Shivani; Ly, K Ina; Fayad, Laura M; Fisher, Michael J; Lessing, Andrés J; Berg, Dale J; Salamon, Johannes M; Mautner, Victor-Felix; Babovic-Vuksanovic, Dusica; Dombi, Eva; Harris, Gordon; Plotkin, Scott R; Blakeley, Jaishri; REiNS International Collaboration.
in: NEUROLOGY, Jahrgang 97, Nr. 7 Suppl 1, 17.08.2021, S. S111-S119.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Imaging Evaluation of Plexiform Neurofibromas in Neurofibromatosis Type 1: A Survey-Based Assessment
AU - Ahlawat, Shivani
AU - Ly, K Ina
AU - Fayad, Laura M
AU - Fisher, Michael J
AU - Lessing, Andrés J
AU - Berg, Dale J
AU - Salamon, Johannes M
AU - Mautner, Victor-Felix
AU - Babovic-Vuksanovic, Dusica
AU - Dombi, Eva
AU - Harris, Gordon
AU - Plotkin, Scott R
AU - Blakeley, Jaishri
AU - REiNS International Collaboration
N1 - © 2021 American Academy of Neurology.
PY - 2021/8/17
Y1 - 2021/8/17
N2 - OBJECTIVE: To assess imaging utilization practices across clinical specialists in neurofibromatosis type 1 (NF1) for the evaluation of symptomatic and asymptomatic children and adults with or without plexiform neurofibromas (PN).METHODS: An institutional review board-exempt survey was administered to medical practitioners caring for individuals with NF1 at the Response Evaluation in Neurofibromatosis and Schwannomatosis (REiNS) meeting in September 2019. The survey included questions on respondent demographic data (9 questions), type of imaging obtained for asymptomatic (4 questions) and symptomatic (4 questions) people with and without PN, and utilization of diffusion-weighted imaging (2 questions).RESULTS: Thirty practitioners participated in the survey. Most were academic neuro-oncologists at high-volume (>10 patients/week) NF1 centers. Of 30 respondents, 26 had access to whole-body MRI (WB-MRI). The most common approach to an asymptomatic person without PN was no imaging (adults: 57% [17/30]; children: 50% [15/30]), followed by a screening WB-MRI (adults: 20% [6/30]; children: 26.7% [8/30]). The most common approach to a person with symptoms or known PN was regional MRI (adults: 90% [27/30]; children: 93% [28/30]), followed by WB-MRI (adults: 20% [6/30]; children: 36.7% [11/30]). WB-MRI was most often obtained to evaluate a symptomatic child with PN (37% [11/30]).CONCLUSIONS: More than 90% of practitioners indicated they would obtain a regional MRI in a symptomatic patient without known or visible PN. Otherwise, there was little consensus on imaging practices. Given the high prevalence of PN and risk of malignant conversion in this patient population, there is a need to define imaging-based guidelines for optimal clinical care and the design of future clinical trials.
AB - OBJECTIVE: To assess imaging utilization practices across clinical specialists in neurofibromatosis type 1 (NF1) for the evaluation of symptomatic and asymptomatic children and adults with or without plexiform neurofibromas (PN).METHODS: An institutional review board-exempt survey was administered to medical practitioners caring for individuals with NF1 at the Response Evaluation in Neurofibromatosis and Schwannomatosis (REiNS) meeting in September 2019. The survey included questions on respondent demographic data (9 questions), type of imaging obtained for asymptomatic (4 questions) and symptomatic (4 questions) people with and without PN, and utilization of diffusion-weighted imaging (2 questions).RESULTS: Thirty practitioners participated in the survey. Most were academic neuro-oncologists at high-volume (>10 patients/week) NF1 centers. Of 30 respondents, 26 had access to whole-body MRI (WB-MRI). The most common approach to an asymptomatic person without PN was no imaging (adults: 57% [17/30]; children: 50% [15/30]), followed by a screening WB-MRI (adults: 20% [6/30]; children: 26.7% [8/30]). The most common approach to a person with symptoms or known PN was regional MRI (adults: 90% [27/30]; children: 93% [28/30]), followed by WB-MRI (adults: 20% [6/30]; children: 36.7% [11/30]). WB-MRI was most often obtained to evaluate a symptomatic child with PN (37% [11/30]).CONCLUSIONS: More than 90% of practitioners indicated they would obtain a regional MRI in a symptomatic patient without known or visible PN. Otherwise, there was little consensus on imaging practices. Given the high prevalence of PN and risk of malignant conversion in this patient population, there is a need to define imaging-based guidelines for optimal clinical care and the design of future clinical trials.
KW - Adolescent
KW - Adult
KW - Child
KW - Humans
KW - Magnetic Resonance Imaging/methods
KW - Male
KW - Middle Aged
KW - Neurilemmoma/pathology
KW - Neurofibroma, Plexiform/diagnosis
KW - Neurofibromatoses/pathology
KW - Neurofibromatosis 1/diagnosis
KW - Skin Neoplasms/pathology
KW - Surveys and Questionnaires
KW - Young Adult
U2 - 10.1212/WNL.0000000000012437
DO - 10.1212/WNL.0000000000012437
M3 - SCORING: Journal article
C2 - 34230200
VL - 97
SP - S111-S119
JO - NEUROLOGY
JF - NEUROLOGY
SN - 0028-3878
IS - 7 Suppl 1
ER -