Cerebellopontine angle epidermoid cyst [Neurosurgery Wiki]

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====== Cerebellopontine angle epidermoid cyst ======
see also [[Cerebellopontine angle dermoid cyst]].

===== Epidemiology =====

Epidermoid cysts are the third most of the [[cerebellopontine angle tumor]]s.

The [[cerebellopontine angle]] [[epidermoid cyst]] account for 3-6% of [[cerebellopontine angle tumor]]s. Comparatively, [[vestibular schwannoma]]s, the most common CPA angle tumor, account for 85%.

Men and women are equally affected and the symptoms usually arise between the mid-20’s and early 50’s
((Fleming JF, Botterell EH. Cranial dermoid and epidermoid tumors. Surg Gynecol Obstet. 1959;109:403–411.))
with a mean age of 38.8 years at presentation
((Fawcitt RA, Isherwood I. Radiodiagnosis of intracranial pearly tumours with particular reference to the value of computer tomography. Neuroradiology. 1976;11:235–242.)).
===== Pathophysiology =====
Although several mechanisms for cranial nerve dysfunction due to these tumors have been proposed.

Hasegawa et al. report the first direct evidence of etiology of cranial nerve dysfunction caused by cerebellopontine angle epidermoid tumors. Young age and rapidly progressive neurological deficit might be the characteristics for strangulation of the affected nerves by the cyst capsule
((Hasegawa M, Nouri M, Nagahisa S, Yoshida K, Adachi K, Inamasu J, Hirose Y,
Fujisawa H. Cerebellopontine angle epidermoid cysts: clinical presentations and
surgical outcome. Neurosurg Rev. 2015 Nov 14. [Epub ahead of print] PubMed PMID: 
26566990.
)).

===== Clinical features =====
CPA epidermoid cysts can compress the surrounding [[cranial nerve]]s, [[brainstem]], and [[cerebellum]]. [[Ataxia]] and cranial nerve palsies often result
((Berger M, Wilson C. Epidermoid cysts of the posterior fossa. J Neurosurg. 1985;62:214–219.)).
----
Thirty cases of cerebellopontine angle epidermoid cysts treated over a period of 20 years were reviewed with regard to their clinical features, the pathophysiology of their symptoms and their management. The predominating symptoms were related to the 7th and 8th cranial nerves and headaches. The signs and symptoms were present for an average period of 4 months. It was not always possible to determine if the signs and symptoms were due to local involvement by the epidermoid, increased intracranial pressure, or both
((deSouza CE, deSouza R, da Costa S, Sperling N, Yoon TH, Abdelhamid MM, Sharma 
RR, Goel A. Cerebellopontine angle epidermoid cysts: a report on 30 cases. J
Neurol Neurosurg Psychiatry. 1989 Aug;52(8):986-90. PubMed PMID: 2795068; PubMed 
Central PMCID: PMC1031839.
)).
===== Diagnosis =====

see [[Intracranial epidermoid cyst diagnosis]]

----
{{cerebelloponineangleepidermoidcyst.jpg?200}}

===== Differential diagnosis =====

{{::mridwiepidermoidcyst.png?300|}}

[[Cerebellopontine angle epidermoid cyst]] may mimic an [[cerebellopontine angle arachnoid cyst]], but are high signal on [[DWI]] MRI.
===== Treatment =====
see [[Cerebellopontine angle epidermoid cyst treatment]].



===== Outcome =====
The extent of tumor removal had no effect on the risk of recurrence, and thus it may be acceptable to leave tumor capsule fragments adhering closely to nerves, vessels, or brainstem. During long-term follow-up, resolution or improvement of present preoperatively and new postoperative neurological deficits may be expected in most patients
((Czernicki T, Kunert P, Nowak A, Wojciechowski J, Marchel A. Epidermoid cysts
of the cerebellopontine angle: Clinical features and treatment outcomes. Neurol
Neurochir Pol. 2016;50(2):75-82. doi: 10.1016/j.pjnns.2015.11.008. Epub 2015 Dec 
11. PubMed PMID: 26969562.
)).
===== Case series =====

[[Cerebellopontine angle epidermoid cyst case series]].

===== Case reports =====

[[Cerebellopontine angle epidermoid cyst case reports]].

===== References =====