Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 May 1;33(3):803-808.
doi: 10.1097/SCS.0000000000008381. Epub 2021 Dec 2.

Challenging Management of Plexiform Schwannoma and Plexiform Neurofibroma

Taija K Nicoli  1 , Riste Saat  2   3 , Jussi Tarkkanen  4 , Ilpo Kinnunen  5 , Antti A Mäkitie  1   6 , Jussi Jero  1
Affiliations

Challenging Management of Plexiform Schwannoma and Plexiform Neurofibroma

Taija K Nicoli et al. J Craniofac Surg. .

Abstract

Plexiform variants of neurofibromas and schwannomas are rare and typically arise in superficial soft tissues in the head and neck region. The treatment of these tumors is challenging and no generally accepted guidelines exist for their optimal management. The purpose of this study was to review the management and longterm prognosis of head and neck plexiform neurofibromas and schwannomas at 2 tertiary care academic hospitals in Finland over a 31-year period. The pathology files were searched for plexiform neurofibromas and schwannomas between the years 1990 and 2020. The case notes were reviewed for full management details. Two plexiform schwannomas and 6 plexiform neurofibromas were identified. Five of the 6 plexiform neurofibromas were managed operatively. All patients with a surgically managed plexiform neurofibroma underwent multiple operations. Sclerotherapy abolished 1 patient's cutaneous plexiform neurofibromas. The management of plexiform neurofibromas and plexiform schwannomas remains challenging. Sclerotherapy may offer a promising management option for cutaneous plexiform neurofibromas.

PubMed Disclaimer

Conflict of interest statement

The authors report no conflicts of interest.

References

    1. The WHO Classification of Tumors Editorial Board. WHO Classification of Tumors Soft Tissue and Bone. 5th ed.Lyon, France: IARC Press; 2020.
    1. Chikkannaiah P, Boovalli MM, Nathiyal V, et al. Morphological spectrum of peripheral nerve sheath tumors: an insight into World Health Organization 2013 classification. J Neurosci Rural Pract 2016; 7:346–354.
    1. Berg JC, Scheithauer BW, Spinner RJ, et al. Plexiform schwannoma: a clinicopathologic overview with emphasis on the head and neck region. Hum Pathol 2008; 39:633–640.
    1. Mobashir MK, Mohamed AE, El-Anwar MW, et al. Massive plexiform neurofibroma of the head and larynx. Int Arch Otorhinolaryngol 2015; 19:349–353.
    1. Chee DW, Peh WC. Shek TW Pictorial essay: imaging of peripheral nerve sheath tumours. Can Assoc Radiol J 2011; 62:176–182.