How effective are noninvasive tests for diagnosing malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1? Diagnosing MPNST in NF1 patients

Background. Distinguishing between benign and malignant peripheral nerve sheath tumors (MPNSTs) in neurofibromatosis 1 (NF1) patients prior to excision can be challenging. How can MPNST be most accurately diagnosed using clinical symptoms, magnetic resonance imaging (MRI) findings (tumor size, depth, and necrosis), positron emission tomography (PET) measures (SUV_peak, SUV_max, SUV_{max tumor}/SUV_{mean liver}, and qualitative scale), and combinations of the above? Methods. All NF1 patients who underwent PET imaging at our institution (January 1, 2007-December 31, 2016) were included. Medical records were reviewed for clinical findings; MR images and PET images were interpreted by two fellowship-trained musculoskeletal and nuclear medicine radiologists, respectively. Receiver operating characteristic (ROC) curves were created for each PET measurement; the area under the curve (AUC) and thresholds for diagnosing malignancy were calculated. Logistic regression determined significant predictors of malignancy. Results. Our population of 41 patients contained 34 benign and 36 malignant tumors. Clinical findings did not reliably predict MPNST. Tumor depth below fascia was highly sensitive; larger tumors were more likely to be malignant but without a useful cutoff for diagnosis. Necrosis on MRI was highly accurate and was the only significant variable in the regression model. PET measures were highly accurate, with AUCs comparable and cutoff points consistent with prior studies. A diagnostic algorithm was created using MRI and PET findings. Conclusions. MRI and PET were more effective at diagnosing MPNST than clinical features. We created an algorithm for preoperative evaluation of peripheral nerve sheath tumors in NF1 patients, for which additional validation will be indicated.