Last Updated: March 27, 2020
- Benign ectodermal inclusion cysts of congenital or acquired etiology
- Most common congenital intracranial tumor (~1%)
- Congenital: Arise from ectodermal epithelium in the 3rd to 5th weeks of embryogenesis during the process of neural tube closure
- May be seen in association with dermal sinus tracts or vertebral anomalies
- Rare acquired lesions are thought to be post-traumatic in etiology, often spinal in location when secondary to lumbar punctures
- Epidermoid cyst growth by desquamation and formation of cholesterol/keratin crystals
- Gross pathologic features include shiny or “pearly” appearance within an outer connective tissue wall
- Cyst contains solid cholesterol crystals and keratin
- Patient ages 20-60 years most common
- No gender predilection
- Presenting signs and symptoms depend on tumor size and location
- Headache, cranial neuropathies (cranial nerves 5, 7, and 8 most common)
- Cyst rupture may cause chemical meningitis
- Often asymptomatic
- Malignant degeneration into squamous cell carcinoma is rare
- Treatment: microsurgical resection; gross total resection is often difficult because of involvement of adjacent structures and specifically attachment of the tumor pearls to the cerebrovascular structures
- Prognosis: recurrence common with incomplete tumor resection or secondary to cerebrospinal spinal fluid (CSF) seeding; malignant degeneration into squamous cell carcinoma is rare
- Most cases (90%) are intradural and located within the basal cisterns (cerebellopontine angle, fourth ventricle, parasellar)
- Minority of cases (10%) are extradural and may arise within the bony calvarium (diploic space) or spine
- Variable size, round or lobulated mass
- Slow but insinuating or invaginating pattern of growth, often encases or surrounds adjacent nerves and vasculature
Computed Tomography (CT)
- Well-defined, usually hypodense mass similar in density to CSF
- <5% appear hyperdense, often due to proteinaceous, iron-rich, or hemorrhagic contents
- Calcifications are common
- Typically do not enhance on contrast enhanced CT
Magnetic Resonance Imaging (MRI)
- Signal intensity typically greater than CSF, but less than brain parenchyma
- Highest signal often seen at the margin of the tumor
- Signal intensity greater than brain parenchyma indicates a “white epidermoid,” brighter because of its higher degree of fatty components or blood products
- Rare hypointense “black epidermoids” caused by low triglyceride and unsaturated fatty acid contents
- Iso- to hyperintense to CSF
- Rare hypointense signal due to iron content or Ca++
- FLAIR: intermediate to hyperintense due to incomplete dropout of fluid signal
- DWI: demonstrates often markedly increased signal (restricts) with corresponding ADC value near that of brain parenchyma (in contrast to an arachnoid cyst which will not restrict)
- T1WI+C: typically nonenhancing, although minimal enhancement at the margin of the tumor may be seen; significant enhancement may suggest malignant degeneration
- MRI with FLAIR and DWI/ADC
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD
Gao PY, Osborn AG, Smirniotopoulos JG, et al. Radiologic-pathologic correlation: epidermoid tumor of the cerebellopontine angle. AJNR Am J Neuroradiol 1992;13:865–872.
Mohanty A, Venkatrama SK, Rao BR, et al. Experience with cerebellopontine angle epidermoids. Neurosurgery. 1997; 40:24–30.
Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier, 2016.
Ren X, et al: Clinical, radiological, and pathological features of 24 atypical intracranial epidermoid cysts. J Neurosurg. 2012;116:611-621.
Tampieri D, Melanson D, Ethier R. MR imaging of epidermoid cysts. AJNR Am J Neuroradiol. 1989; 10:351–356.
Velamati R, et al: Meningitis secondary to ruptured epidermoid cyst: case-based review. Pediatr Ann. 2013;42:248-251.
Vellutini EA, et al: Malignant transformation of intracranial epidermoid cyst. Br J Neurosurg. 2014;28:507-509.
Please login to post a comment.