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BACKGROUND Understanding the natural history of non-malignant peripheral nerve sheath tumors (PNSTs) in neurofibromatosis type 1 (NF1) is critical to optimal clinical care and the development of meaningful clinical trials. METHODS We longitudinally analyzed growth of plexiform neurofibromas (PNs) and of PNSTs with distinct nodular appearance (distinct nodular lesions/DNLs) using volumetric MRI analysis in patients enrolled on a natural history study (NCT00924196). RESULTS DNLs were observed in 58/122 (45.6%) patients (median 2 DNLs/pat