Case Report

A Case of Cerebellopontine Angle Epidermoid Cyst Presenting as Trigeminal Neuropathy

Sadat Muzammil,
MBBS,

and King Sun Leong,

MBBS, FRCP

Abstract: We present the case of a 35-year-old female who had paraesthesia and numbness on the left side of her face. Initially the ophthalmic division and maxillary divisions on the trigeminal nerve were involved, but later involved the mandibular division as well. On MRI, a cerebellopontine angle (CPA) epidermoid cyst was detected. After 18 months of follow up, her symptoms remain stable and she is being managed conservatively. Key Words: cerebellopontine angle tumor, differential diagnosis, MRI, treatment options, trigeminal nerve

pidermoid cysts are congenital lesions arising from ectopic ectodermal cells that are retained within the neural groove at the time of closure at 35 weeks gestation. They grow slowly and present in adulthood. They contain keratin, cellular debris and cholesterol, and are lined with stratified squamous epithelium. They account for 2 6% of all intracranial tumors and less than 1% of all intraspinal tumors. Epidermoid cysts of the cerebellopontine angle (CPA) can affect an isolated nerve or more than one nerve. In either situation the signs and symptoms will vary according to the nerve(s) involved. This is an interesting case where subtle features were later diagnosed as a CPA tumor. Here we discuss the different presenting features, early diagnostic points, surgical and medical management options of an epidermoid cyst and the differential diagnosis of a CPA tumor.

Case Report
A 35-year-old female paramedic presented to a general medical clinic with a four-month history of left-sided altered facial sensation. The patient had

paraesthesia and numbness over the left side of her face in an ophthalmic and maxillary distribution, which subsequently developed to involve all three divisions of the left trigeminal nerve. She also noticed grittiness in her left eye, but no dryness. She saw her general practitioner who referred her to a medical clinic for detailed assessment. Apart from a moderate to high alcohol intake on weekends, there were no significant findings in the patients history. On examination there was a mild decrease in sensation over all three divisions of the left trigeminal nerve, but with an intact corneal reflex and there was no Horner syndrome. She denied any facial pain or headaches. There was no vertigo, dizziness or tinnitus and no hearing loss on examination. The rest of the cranial nerves were intact and cerebellar examination was normal. Blood tests were also normal. An MRI scan of the brain was requested. A T2-weighted MRI showed a 1.5 x 1.0 cm mass (Fig. 1) lying adjacent to the left side of the pons. This was compressing the left trigeminal nerve with characteristics consistent with an epidermoid cyst. The patient was referred to the regional center for neurology and neurosurgery. A year later, she has had follow up in the neurosurgery clinic with an MRI brain scan following gadolinium which showed a nonenhancing cyst measuring 2.5 cm anteroposteriorly. It was slightly hypointense to cerebrospinal fluid (CSF) on T2 imaging, hyperintense to CSF and brain on FLAIR imaging and
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Key Points
From the Department of Medicine, Wirral University Teaching Hospital, Wirral, England, United Kingdom. Reprint requests to King Sun Leong, MBBS, Department of Medicine, Wirral University Teaching Hospital, Wirral, Merseyside CH59 5PE, England, United Kingdom. Email: kleong@nhs.net Accepted January 14, 2009. Copyright 2009 by The Southern Medical Association 0038-4348/0 2000/10200-0534

Epidermoid cysts are congenital lesions which grow slowly and present in adulthood. They account for 2 6% of all intracranial tumors and less than 1% of all intraspinal tumors. Epidermoid cysts of the cerebellopontine angle (CPA) can affect an isolated nerve or more than one nerve. The signs and symptoms vary according to the nerve(s) involved.

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showed a degree of restrict diffusion to CSF on diffusion weighted imaging. It showed no enhancement following gadolinium, consistent with an epidermoid cyst. (Figs. 2 6). As the patient was otherwise well, a conservative approach was taken. However, the patient remained stable with no progression of her condition 18 months after diagnosis. She continues to be managed conservatively with annual follow up.

Discussion
The most common symptoms of CPA epidermoid cysts include hearing loss, tinnitus, dizziness and unsteadiness1. Other less common presenting symptoms are headaches, trigeminal neuralgia, facial numbness, facial spasms, facial palsy, impaired taste and intention tremors. Epidermoid cysts also occur in the fourth ventricle (17%) and the sellar and/or parasellar regions (10%15%). Less common locations include the cerebral hemispheres or brainstem. Ten percent of epidermoid cysts are extradural, located in the skull or spine. Suspicion of a CPA tumor should arise when a young and otherwise generally healthy patient presents with new onset localized cranial nerve involvement, mostly involving the 5th, 7th or 8th cranial nerve. Patients often do not present until the second to fourth decade of life. Large CPA tumors can present with features of raised intracranial pressure. This happens when the tumor is blocking the aqueduct, leading to distortion of the ventricular system in the brain.2 Multiple sclerosis, infarction and gliomas are the most common abnormalities in the brain stem leading to trigeminal neu-

Fig. 2 T2 FLAIR. ropathy. The most common cisternal cause is neurovascular compression, followed by acoustic and trigeminal schwannomas, meningiomas, lipomas, and metastases. Trigeminal neuropathy arising from the Meckel cave and cavernous sinus is frequently due to meningiomas, trigeminal schwannomas, epidermoid cysts, metastases, pituitary adenomas, and aneurysms3. Trigeminal neuropathy may be the initial symptom of a CPA epidermoid cyst, particularly in young patients; this was also the presenting feature of our patient. Radiologically, epidermoid cysts are characteristically hypodense nonenhancing lesions on CT scans, while on MRI they exhibit long T1 and T2 relaxation times.4 A

Fig. 1 T2 FLAIR.
Southern Medical Journal Volume 102, Number 5, May 2009

Fig. 3 T1 Precontrast.

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Fig. 4 Precontrast gadolinium. conservative approach is indicated for patients in whom the capsule is adherent to the brain stem and the cranial nerves, or when patients are clinically stable. A retrolabyrinthine approach (RLA) combined with endoscopy should be considered the standard approach for excision of epidermoid cysts. Extension of this approach into a translabyrinthine approach (TLA) or transcochlear approach will depend on preoperative difficulties5. Surgical intervention has been performed when tumors were large, ranging from 2 x 3 cm to 6 x 6 cm; smaller tumors with minimal features are preferably treated conservatively. Cranial nerve dysfunction and aseptic meningitis are the most common operative complications.

Fig. 6 T2.

Conclusion
Neurological signs and symptoms can be vague in a young healthy person. Isolated cranial nerve(s) involvement merits detailed investigations. Our patient had been experiencing neurological signs and symptoms for several months before being referred to the hospital. Early referral is essential in such conditions for further assessment. Modern radiological modalities are very helpful in diagnosing complicated cases. This helps in referring the patient to an appropriate specialty. Patients who show nonprogressive clinical features and have stable MRI scans can be managed conservatively with regular follow ups.

Acknowledgment
We thank Dr. Sacha Niven, consultant neuroradiologist, Walton Centre of Neurology and Neurosurgery, for the radiological advice in this case.

References
1. Yasargil MG, Abernathey CD, Sarioglu AC. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989; 24:561567. 2. deSouza CE, deSouza R, da Costa S, et al. Cerebellopontine angle epidermoid cysts: a report on 30 cases. J Neurol Neurosurg Psychiatry 1989;52:986 990. 3. Nemzek WR. The trigeminal nerve. Top Magn Reson Imaging 1996;8: 132154. 4. Meng L, Yuguang L, Feng L, et al. Cerebellopontine angle epidermoids presenting with trimeminal neuralglia. Clin Neurosci 2005;12: 784 786. 5. Darrouzet V, Franco-Vidal V, Hilton M, et al. Surgery of cerebellopontine angle epidermoid cysts: role of the widened retrolabyrinthine approach combined with endoscopy. Otolaryngol Head Neck Surg 2004; 131:120 125.

Fig. 5 Diffusion weighted imaging.

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