1. Imaging Evaluation of Plexiform Neurofibromas in Neurofibromatosis Type 1
- Author
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Johannes Salamon, Dale J. Berg, Shivani Ahlawat, Michael Fisher, Dusica Babovic-Vuksanovic, Jaishri O. Blakeley, K. Ina Ly, Gordon J. Harris, Andrés J. Lessing, Laura M. Fayad, Scott R. Plotkin, Eva Dombi, and Victor-Felix Mautner
- Subjects
Adult ,Male ,Pediatrics ,medicine.medical_specialty ,Neurofibromatosis 1 ,Skin Neoplasms ,Adolescent ,Neurofibromatoses ,MEDLINE ,Asymptomatic ,Imaging ,Young Adult ,Surveys and Questionnaires ,Humans ,Medicine ,Clinical care ,Neurofibromatosis ,Child ,Schwannomatosis ,Neurofibroma, Plexiform ,High prevalence ,business.industry ,Middle Aged ,medicine.disease ,Magnetic Resonance Imaging ,Clinical trial ,Patient population ,Neurology (clinical) ,medicine.symptom ,business ,Neurilemmoma - Abstract
ObjectiveTo 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).MethodsAn 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).ResultsThirty 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]).ConclusionsMore 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.
- Published
- 2021
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