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Evaluation of the most commonly used (semi-)quantitative parameters of 18F-FDG PET/CT to detect malignant transformation of neurofibromas in neurofibromatosis type 1

Brinkman, Marloesa; Jentjens, Sanderb,f; Boone, Kittyb; Anten, Moniquea,c; Stumpel, Constance T.R.M.c,d; Nelemans, Patty J.e; van Kroonenburgh, Marinus J.P.G.b,c

doi: 10.1097/MNM.0000000000000889

In patients with neurofibromatosis type 1, transformation of neurofibromas into a malignant peripheral nerve sheath tumor (MPNST) is a severe complication of the disease. Fluorine-18-fluorodeoxyglucose PET/computed tomography (PET/CT) is a viable option for detecting malignant tumors in neurofibromatosis type 1 patients. The aim of this review was to assess the diagnostic performance of the most frequently used parameters of PET/CT in detecting MPNST. An extensive computer search was performed using the Cochrane Library, Pubmed, and Medline/Embase databases. Two reviewers independently extracted data of relevant studies and assessed the methodological quality (QUADAS-2). The diagnostic performance of PET/CT parameters in individual studies was determined by calculating a diagnostic odds ratio (DOR) using the absolute numbers of true-positive, true-negative, false-positive, and false-negative test results. A total of eight studies were included, of which three evaluated the standardized uptake value as a diagnostic parameter, two assessed the tumor-to-liver (T/L) ratio, and three articles described both parameters. The cut-off values for maximum standardized uptake value (SUVmax) ranged from 3.2 to 4.5; for the T/L ratio, the cut-off values were between 1.0 and 4.3. The sensitivity and specificity ranged from 90 to 100% and from 80 to 100%, respectively (SUVmax). T/L ratios were associated with 92–100% sensitivity and 72–94% specificity. The corresponding DORs ranged from 57 to 145 (SUVmax) and 35 to 655 (T/L ratio). Both the SUV and the T/L ratio are associated with high sensitivity combined with acceptable specificity in detecting MPNST. There is a tendency toward higher DORs using the T/L ratio, but the number of studies is limited.

aDepartment of Neurology

bDepartment of Radiology and Nuclear Medicine

cNeurofibromatosis Expert Team

dDepartment of Clinical Genetics

eDepartment of Epidemiology, Maastricht University Medical Centre, Maastricht

fDepartment of Nuclear Medicine, Catharina Hospital Eindhoven, Eindhoven, The Netherlands

Correspondence to Marinus J.P.G. van Kroonenburgh, MD, PhD, Department of Radiology and Nuclear Medicine, Maastricht University Medical Centre, P. Debyelaan 25, 6229 HX Maastricht, The Netherlands Tel: +31 433 874 746; fax: +31 433 876 746; e-mail:

Received May 8, 2018

Received in revised form July 4, 2018

Accepted July 16, 2018

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