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
Case Reports
. 2019 Apr 26;14(1):e9-e13.
doi: 10.1055/s-0039-1685457. eCollection 2019 Jan.

Schwannomatosis of the Spinal Accessory Nerve: A Case Report

Ramin A Morshed  1 , Anthony T Lee  1 , Young M Lee  1 , Cynthia T Chin  2 , Line Jacques  1
Affiliations
Case Reports

Schwannomatosis of the Spinal Accessory Nerve: A Case Report

Ramin A Morshed et al. J Brachial Plex Peripher Nerve Inj. .

Abstract

Schwannomatosis is a distinct syndrome characterized by multiple peripheral nerve schwannomas that can be sporadic or familial in nature. Cases affecting the lower cranial nerves are infrequent. Here, the authors present a rare case of schwannomatosis affecting the left spinal accessory nerve. Upon genetic screening, an in-frame insertion at codon p.R177 of the Sox 10 gene was observed. There were no identifiable alterations in NF1, NF2, LZTR1, and SMARCB1. This case demonstrates a rare clinical presentation of schwannomatosis in addition to a genetic aberration that has not been previously reported in this disease context.

Keywords: diffusion tensor imaging; neck mass; schwannoma; schwannomatosis; spinal accessory nerve tumor.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest None declared.

Figures

Fig. 1
Fig. 1
( A–C ) Positron emission tomography–computed tomography (PET CT) was performed, which demonstrated increased fluoro-2-deoxy-d-glucose uptake in both masses (standardized uptake value of 7.3 for the larger mass and 2.7 for the smaller mass).
Fig. 2
Fig. 2
Magnetic resonance imaging (MRI) neurogram demonstrated a larger 4.6 × 3.2 × 2.5 cm mass deep to the left sternocleidomastoid muscle just below the angle of the mandible (A–C) and a bilobed 2.4 × 2.2 × 1.3 cm mass in the left posterior supraclavicular region (D–F) .
Fig. 3
Fig. 3
Further imaging characterization of lesions. (A) Diffusion tensor imaging with tractography demonstrated abnormal thickened nerve fibers coursing through the two spinal accessory nerve tumors. (B) Axial diffusion weight imaging (left) and apparent diffusion coefficient (ADC) images (right) of the lesions. ADC values were 1.3 × 10 −6 and 1.8 × 10 −6 mm 2 /second for the larger and smaller masses, respectively.
Fig. 4
Fig. 4
Intraoperative findings. (A) Two separate incisions were required to remove both lesions. (B) The smaller bilobed lesion mass was accessed through the posterior triangle of the neck and was located on the distal spinal accessory nerve. (C) The larger more proximal mass was approached medial to the sternocleidomastoid muscle in the upper neck. Both masses underwent a gross total resection.
See this image and copyright information in PMC

References

    1. International Agency for Research on Cancer.WHO Classification of Tumors of the Central Nervous System. 4th ed Geneva: World Health Organization; 2016
    1. MacCollin M, Chiocca E A, Evans D G et al. Diagnostic criteria for schwannomatosis. Neurology. 2005;64(11):1838–1845. - PubMed
    1. Evans D G, Bowers N L, Tobi S et al. Schwannomatosis: a genetic and epidemiological study. J Neurol Neurosurg Psychiatry. 2018;89(11):1215–1219. - PubMed
    1. Kresak J L, Walsh M. Neurofibromatosis: a review of NF1, NF2, and Schwannomatosis. J Pediatr Genet. 2016;5(02):98–104. - PMC - PubMed
    1. Huang J H, Simon S L, Nagpal S, Nelson P T, Zager E L.Management of patients with schwannomatosis: report of six cases and review of the literature Surg Neurol 20046204353–361., discussion 361 - PubMed

Publication types