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Plexiform Tumors

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Diagnostic Assessment and Treatment of Peripheral Nerve Tumors

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Abstract

Plexiform tumours are pathognomonic variants of peripheral nerve tumours in NF. Two forms of plexiform neoplasms are encountered: plexiform schwannomas (PSs) and plexiform neurofibromas (PNs). The former are rare. They only account for 5% of all schwannomas; they usually arise in the head or neck of the patients affected by NF2 or schwannomatosis. They never undergo malignant transformation.

Plexiform neurofibromas are found in up to 50% of NF1 patients. Usually diagnosed during childhood, they present variable behaviour; some lesions tend to remain stable, and others may constantly grow and eventually become symptomatic. Differently from their schwannoma counterpart, PNs frequently undergo malignant transformation.

Surgery is the current mainstay of treatment for plexiform neurofibromas; however, they can rarely be fully resected due to their involvement of critical structures. Hence surgery frequently results in gross debulking only, predisposing to regrowth. The limitations of surgery are the motivators for highly active efforts to discover drugs able to arrest or reverse the growth of these tumours, especially in children.

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Authors and Affiliations

  1. WFNS Peripheral Nerve Surgery Committee, Department of Neurosurgery, Mediclinic Parkview Hospital, Dubai, UAE

    Debora Garozzo

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  1. Debora Garozzo
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Correspondence to Debora Garozzo .

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Editors and Affiliations

  1. WFNS Peripheral Nerve Surgery Committee Division of Neurosurgery, Department of Surgery, Gaffrée e Guinle University Hospital (HUGG) School of Medicine Federal University of Rio de Janeiro State (UNIRIO), Rio de Janeiro, Rio de Janeiro, Brazil

    Fernando Guedes

  2. WFNS Peripheral Nerve Surgery Committee Department of Neurosurgery, University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA

    Eric L. Zager

  3. WFNS Peripheral Nerve Surgery Committee Department of Neurosurgery, Mediclinic Parkview Hospital, Dubai, United Arab Emirates

    Debora Garozzo

  4. WFNS Peripheral Nerve Surgery Committee, Faculty of Medicine University of Belgrade, Clinic for Neurosurgery, University Clinical Center of Serbia, Department of Peripheral Nerve Surgery, Functional Neurosurgery and Pain Management Surgery, Belgrade, Serbia

    Lukas Rasulic

  5. WFNS Peripheral Nerve Surgery Committee Peripheral Nerve & Brachial Plexus Surgery Program Department of Neurosurgery, University of Buenos Aires School of Medicine, Buenos Aires, Argentina

    Mariano Socolovsky

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Garozzo, D. (2021). Plexiform Tumors. In: Guedes, F., Zager, E.L., Garozzo, D., Rasulic, L., Socolovsky, M. (eds) Diagnostic Assessment and Treatment of Peripheral Nerve Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-77633-6_28

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