Intracranial epidermoid cyst

Intracranial epidermoid cyst

Epidemiology

Intracranial epidermoid cysts, are rare congenital lesions originating from the ectoderm that constitute 0.3 to 1.8 % of all intracranial neoplasms 1) 2).

The most common sites include

a) suprasellar: commonly produce bitemporal hemianopsia and optic atrophy, and only occasionally pituitary (endocrine) symptoms (including DI)

b) sylvian fissure: may present with seizures

c) cerebellopontine angle (CPA): may produce trigeminal neuralgia, especially in young patient

d) basilar-posterior fossa: may produce lower cranial nerve findings, cerebellar dysfunction, and/or corticospinal tract abnormalities

e) within the ventricular system: occur within the 4th ventricle more commonly than any other

Approximately half of these cysts are located at the cerebellopontine angle (CPA), although they can also be found in the parasellar regions, 4th ventricle, cerebellum, cerebral hemispheres, brainstem, and lateral ventricles.

see Cavernous sinus epidermoid

Cerebellopontine angle epidermoid cyst

Giant intracranial epidermoid.

Intradiploic epidermoid

Intraventricular epidermoid

Middle fossa epidermoid cyst

Pineal epidermoid cyst

Posterior fossa epidermoid cyst

Sylvian fissure epidermoid cyst.

Etiology

Intracranial epidermoid cysts, also known as primary cholesteatomas are considered to arise from epithelial inclusions at the time of neural tube closure or during formation of the secondary cerebral vesicles, and have slow growth rates resembling that of the normal epidermis 3).

Pathology

Pathologically, epidermoid cysts have well-circumscribed, irregular, thin walls with squamous epithelium lining. The epithelium undergoes progressive desquamation and keratin breakdown; therefore, the cystic contents include tissue debris, keratin, water, and solid cholesterol 4).

These tumors are congenital and arise from displaced epithelial tissue between the 3rd and 5th weeks of gestation, when neural tube closure occurs.

These brain lesions have an epithelial lining that encapsulates the tumor, which consists of desquamated epidermal cell debris. Most lesions are solid, but because the desquamated cells contain cholesterol some may have a liquid cystic center. The rate of tumor growth is gradual and linear, resembling the rate of human epidermis turnover, which is unlike the exponential growth of neoplastic lesions 5).

Diagnosis

Treatment

The treatment of choice for epidermoid cyst is a total resection of the tumor. But sometimes total resection is impossible because of anatomical complexity.

A conservative attitude in handling the tumor capsule is common given concerns about capsule adherence to neurovascular structures, and thus the possibility of recurrence is accepted with the intent of minimizing complications 6).

Outcome

Malignant transformation of an EC to squamous-cell carcinoma is rare; only 14 cases have been reported 7).

Case series

Vaz-Guimaraes et al., from Pittsburgh, Houston, and Toronto retrospectively reviewed the medical records of 21 patients who underwent endoscopic endonasal surgery for epidermoid and dermoid cyst resection at the University of Pittsburgh Medical Center between January 2005 and June 2014. Surgical outcomes and variables that might affect the extent of resection and complications were analyzed.

Total resection (total removal of cyst contents and capsule) was achieved in 8 patients (38.1%), near-total resection (total removal of cyst contents, incomplete removal of cyst capsule) in 9 patients (42.9%), and subtotal resection (incomplete removal of cyst contents and capsule) in 4 patients (19%). Larger cyst volume (≥ 3 cm3) and intradural location (15 cysts) were significantly associated with nontotal resection (p = 0.008 and 0.0005, respectively). In the whole series, surgical complications were seen in 6 patients (28.6%). No complications were observed in patients with extradural cysts. Among the 15 patients with intradural cysts, the most common surgical complication was postoperative CSF leak (5 patients, 33.3%), followed by postoperative intracranial infection (4 patients, 26.7%). Larger cysts and postoperative CSF leak were associated with intracranial infection (p = 0.012 and 0.028, respectively). Subtotal resection was marginally associated with intracranial infection when compared with total resection (p = 0.091). All patients with neurological symptoms improved postoperatively with the exception of 1 patient with unchanged abducens nerve palsy.

Endoscopic endonasal approaches may be effectively used for resection of epidermoid and dermoid cysts in carefully selected cases. These approaches are recommended for cases in which a total or near-total resection is possible in addition to a multilayer cranial base reconstruction with vascularized tissue to minimize the risk of intracranial infection 8).

References

1) , 3) , 4)

Kato K, Ujiie H, Higa T, Hayashi M, Kubo O, Okada Y, et al. Clinical presentation of intracranial epidermoids: a surgical series of 20 initial and four recurred cases. Asian J Neurosurg. 2010;5(1):32–40.
2)

Toglia JU, Netsky MG, Alexander E., Jr Epithelial (epidermoid) tumors of the cranium. Their common nature and pathogenesis. J Neurosurg. 1965;23(4):384–93. doi: 10.3171/jns.1965.23.4.0384.
5)

Alvord EC., Jr. Growth rates of epidermoid tumors. Ann Neurol. 1977;2:367–370.
6)

Aboud E, Abolfotoh M, Pravdenkova S, Gokoglu A, Gokden M, Al-Mefty O. Giant intracranial epidermoids: is total removal feasible? J Neurosurg. 2015 Jan 16:1-14. [Epub ahead of print] PubMed PMID: 25594324.
7)

Vellutini EA, de Oliveira MF, Ribeiro AP, Rotta JM. Malignant transformation of intracranial epidermoid cyst. Br J Neurosurg. 2014 Aug;28(4):507-9. doi: 10.3109/02688697.2013.869552. Epub 2013 Dec 18. Review. PubMed PMID: 24345076.
8)

Vaz-Guimaraes F, Koutourousiou M, de Almeida JR, Tyler-Kabara EC, Fernandez-Miranda JC, Wang EW, Snyderman CH, Gardner PA. Endoscopic endonasal surgery for epidermoid and dermoid cysts: a 10-year experience. J Neurosurg. 2018 Mar 1:1-11. doi: 10.3171/2017.7.JNS162783. [Epub ahead of print] PubMed PMID: 29547084.

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