Abstract
PurposeTo determine the metabolically active whole-body tumor volume (WB-MTV) on F-18-fluorodeoxyglucose positron emission tomography/computed tomography (F-18-FDG PET/CT) in individuals with neurofibromatosis type 1 (NF1) using a three-dimensional (3D) segmentation and computerized volumetry technique, and to compare PET WB-MTV between patients with benign and malignant peripheral nerve sheath tumors (PNSTs).Patients and MethodsThirty-six NF1 patients (18 patients with malignant PNSTs and 18 age- and sex-matched controls with benign PNSTs) were examined by F-18-FDG PET/CT. WB-MTV, whole-body total lesion glycolysis (WB-TLG) and a set of semi-quantitative imaging-based parameters were analyzed both on a per-patient and a per-lesion basis.ResultsOn a per-lesion basis, malignant PNSTs demonstrated both a significantly higher MTV and TLG than benign PNSTs (p < 0.0001). On a per-patient basis, WB-MTV and WB-TLG were significantly higher in patients with malignant PNSTs compared to patients with benign PNSTs (p < 0.001). ROC analysis showed that MTV and TLG could be used to differentiate between benign and malignant tumors.ConclusionsWB-MTV and WB-TLG may identify malignant change and may have the potential to provide a basis for investigating molecular biomarkers that correlate with metabolically active disease manifestations. Further evaluation will determine the potential clinical impact of these PET-based parameters in NF1.
Highlights
Neurofibromatosis type 1 (NF1) is a rare hereditary tumor predisposition syndrome caused by a germline mutation in the NF1 tumor suppressor gene [1]
On a per-patient basis, WB-metabolic tumor volume (MTV) and whole-body total lesion glycolysis (WB-total lesion glycolysis (TLG)) were significantly higher in patients with malignant peripheral nerve sheath tumors (PNSTs) compared to patients with benign PNSTs (p < 0.001)
Receiver operating characteristic (ROC) analysis showed that MTV and TLG could be used to differentiate between benign and malignant tumors
Summary
Neurofibromatosis type 1 (NF1) is a rare hereditary tumor predisposition syndrome caused by a germline mutation in the NF1 tumor suppressor gene [1]. Individuals with NF1 may develop a variety of benign and malignant tumors, the most frequent being peripheral nerve sheath tumors (PNSTs) [2]. PNSTs are neoplasms arising from Schwann cells [3]. Called plexiform neurofibromas may undergo transformation into malignant peripheral nerve sheath tumors (MPNSTs) [4]. Patients with NF1 have a lifetime risk of up to 10% for developing MPNSTs [5,6]. Prognosis is usually poor in these rapidly metastasizing neoplasms, underlining the need for early detection of malignant transformation and identification of risk factors
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