Cancer Genetics and Epigenetics 2017, Vol.5, No.5, 25-27
25
A Letter Open Access
A Rare Case of Plexiform Schwannoma Involving Cerebellopontine Angle
Dou Yafang, Liang Zonghui
Department of radiology, Jing’an District Center Hospital of Fudan University, No.259, Xikang Road, Shanghai, China
Corresponding author email
Cancer Genetics and Epigenetics, 2017, Vol.5, No.5 doi
Received: 05 Dec., 2017
Accepted: 13 Dec., 2017
Published: 26 Dec., 2017
Copyright © 2017
Dou and Liang, This is an open access article published under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:
Dou Y.F. and Liang Z.H., 2017, A rare case of plexiform schwannoma involving cerebellopontine angle, Cancer Genetics and Epigenetics, 5(5): 25-27 (doi:
Abstract
Plexiform schwannoma (PS) is an infrequent variety of benign nerve sheath tumor characterized by multi-nodular
(plexiform) architecture and the individual thin capsule. The pre-operative diagnosis of PS is difficult. In this report, we describe a
case of PS presented in the cerebellopontine angle (CPA) space. Our case involved a 32-year-old woman with one-month history of
worsening verbal vagueness associated with unstable gait. MRI revealed a solid-cystic mass with in the left CPA region. The mass
was resected completely, and the diagnosis of PS was confirmed by histopathology. While schwannoma commonly occurs in the
head and neck, CPA space PS is rare, and pre-operative pathological diagnosis of PS is difficult. MRI studies of PS revealed
distinctive features that we found useful in pre-operative diagnosis.
Keywords
Plexiform schwannoma; Cerebellopontine angle; Diagnosis; MRI
Background
Schwannoma is a benign, encapsulated nerve sheath tumour composed of primarily Schwann cells in a poorly
collagenised stroma. Plexiform schwannoma (PS) is an extremely rare and unique variant of Schwann cell
tumours characterized grossly and histologically by multinodular architecture and the individual thin capsule
(Ijichi et al., 2017). It comprises up to 5% of all schwannomas (Kudose et al., 2016). Most cases of PS occur in
the skin and subcutaneous soft tissue, and approximately 20% of PSs are located in the head and neck region
(Berg et al., 2008; Kohyama et al., 2011). In this report, the rare case of PS occurred in the CPA region. This is
thought to be the first case report of PS growing from this region and the facial nerve. We hereby report this case,
illustrating that PS has very few differential features, as well as presenting the characteristics of clinical and
imaging diagnosis.
1 Case Presentation
The patient was a 32-year-old woman in good health. She had a one-month history of worsening verbal vagueness
associated with unstable gait. Following diagnosis, patient was referred to our hospital. Physical examination
revealed positive Romberg’s sign.
Magnetic resonance imaging (MRI) of brain, with and without gadolinium, revealed a mass in the
cerebellopontine angle space (Figure 1(a-f)). It was hypointense on T1-weighted imaging and hyperintense on
T2-weighted imaging with heterogeneous enhancement. Based on imaging characteristics, primary considerations
included schwannoma, or ependymoma. She did not have other problems in his medical or family history. The
tumor was excised completely. During the operation, the mass was visible at subdural and ventral brain stem. It
was an encapsulated tumor mass with a fairly firm and smooth surface. The lesion contains multiple cystic
changed and was pushing the brain stem and cerebellum laterally to the right. It was also revealed that posterior
cranial nerve (facial nerve) went through the mass. The intraoperative pathological diagnosis was plexiform
schwannoma involving the foramina jugulare.
Cyst compartments presented with slight hyperintense signal compared with cerebral spinal fluid (CSF) on
T2-weighted images; and solid compartments showed slight hyper signal intensity on T2-weighted and FLAIR
images compared with parenchyma. Contrast-enhanced T1-weighted images showed heterogeneous enhancement.
