Abstract
Peripheral nerve sheath tumors are a heterogeneous subgroup of soft tissue tumors that either arise from a peripheral nerve or show nerve sheath differentiation. On imaging, direct continuity with a neural structure or location along a typical nerve distribution represents the most important signs to suggest the diagnosis. Ultrasound and magnetic resonance imaging are the best modalities to evaluate these lesions. First, it is necessary to differentiate between a true tumor and a non-neoplastic nerve condition such as a neuroma, peripheral nerve ganglion, intraneural venous malformation, lipomatosis of nerve, or nerve focal hypertrophy. Then, with a combination of clinical features, conventional and advanced imaging appearances, it is usually possible to characterize neurogenic tumors confidently. This article reviews the features of benign and malignant peripheral nerve sheath tumors, including the rare and recently described tumor types. Furthermore, other malignant neoplasms of peripheral nerves as well as non-neoplastic conditions than can mimick neurogenic tumor are herein discussed.
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Key points
• On imaging, direct continuity with a neural structure, location along a typical nerve distribution, fusiform shape, split-fat sign, target sign, and fascicular appearance suggest the diagnosis of peripheral nerve tumor.
• Intraneural perineurioma is an underdiagnosed benign nerve neoplasm that affects children and young adults, and presents characteristic imaging features.
• In the setting of type 1 neurofibromatosis, malignant peripheral nerve sheath tumor must be suspected in case of a distinct growing mass on MR imaging or increased activity on PET.
• Non-neurogenic neoplasms of peripheral nerves (synovial sarcoma, neurolymphoma, and metastatic disease) can mimic peripheral nerve sheath tumor on imaging, and should therefore be included in the differential diagnosis.
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Lefebvre, G., Le Corroller, T. Ultrasound and MR imaging of peripheral nerve tumors: the state of the art. Skeletal Radiol 52, 405–419 (2023). https://doi.org/10.1007/s00256-022-04087-5
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DOI: https://doi.org/10.1007/s00256-022-04087-5