Value of PET in the assessment of patients with neurofibromatosis type I

OBJECTIVE. The objective of our study was to investigate the use of PET in the detection of malignant peripheral nerve sheath tumors (MPNSTs) in patients with neurofibromatosis type 1 (NF1). MATERIALS AND METHODS. Forty-five patients with NF1 who underwent wholebody PET for suspected MPNST based on clinical symptoms or radiologic examinations were ret-respectively evaluated. Ten patients underwent additional carbon-11 (C-11) methionine PET because of equivocal F-18-FDG PET findings or because of a discrepancy between the FDG PET and clinical findings. PET images were evaluated for the distribution and uptake pattern, and the standardized uptake values (SUVs) were obtained. Twenty-seven patients underwent biopsy or surgery of the detected lesions and 18 patients were followed up clinically and with repeat imaging studies. RESULTS. Fifty lesions were identified on FDG PET. There were eight false-positive results and one false-negative on FDG PET. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of FDG PET in detecting MPNSTs in patients with NF1 were 95%, 72%, 71%, 95%, and 82%, respectively. Using C-11 methionine PET in combination with FDG PET reduced the number of false-positive results from eight to two, which increased the specificity from 72% to 91%. In five patients, C-11 methionine FDG PET contributed additional information about nontarget lesions that influenced treatment planning. CONCLUSION. FDG PET is a sensitive technique in the detection of MPNSTs in patients with NF1. The addition of C-11 methionine PET increases specificity in equivocal cases. PET may improve preoperative tumor staging by detecting metastases or second primary tumors, which often are present in patients with NF1.