Epidermoid Tumor: Overview, Symptoms, Diagnosis, and Treatment

Epidermoid tumors—also referred to as epidermoid cysts or epidermoid inclusion tumors—are rare, benign, slow-growing intracranial lesions that originate from ectodermal remnants trapped during early embryonic development. Although histologically non-malignant, they can cause significant neurological symptoms due to their infiltrative growth pattern and proximity to critical neural structures.

What Is an Epidermoid Tumor?

These lesions are lined with keratinizing squamous epithelium and contain accumulated keratin, cholesterol crystals, and lipid debris, giving them a pearly, waxy appearance—hence the nickname “pearly tumors.” Epidermoid tumors expand slowly, often over decades, and tend to grow along the subarachnoid spaces, enveloping cranial nerves and major blood vessels. Although benign, their location within neurosurgically sensitive regions can make complete surgical removal difficult, especially without causing neurological damage.

Common Locations of Epidermoid Tumors

Epidermoid tumors are most frequently found in cerebrospinal fluid–rich spaces and regions of embryological complexity. Common anatomical locations include the cerebellopontine angle (CPA), the temporal lobe region, and the parasellar cisterns. Less commonly, they may be found in the fourth ventricle, prepontine cistern, or even the spinal canal.

Symptoms of Epidermoid Tumor

Since epidermoid tumors grow very slowly, symptoms often go unnoticed for years and become apparent only when adjacent neural structures are compressed. Typical symptoms include:

Hearing Loss and Tinnitus: Frequently due to compression of the vestibulocochlear nerve (cranial nerve VIII), especially in CPA tumors.

Facial Weakness or Numbness: Involvement of cranial nerves V and VII may cause altered facial sensation or facial muscle weakness.

Dizziness, Vertigo, or Imbalance: Resulting from cerebellar or vestibular nerve compression.

Persistent Headaches and Nausea: Often related to increased intracranial pressure or hydrocephalus.

Visual Disturbances: Such as double vision or visual field loss, especially when tumors are located near the parasellar cisterns.

Seizures: More common in supratentorial epidermoids that irritate the cerebral cortex.

Diagnostic Evaluation

Magnetic Resonance Imaging (MRI): MRI is the imaging modality of choice. Key features include:

T1-weighted: Typically hypointense

T2-weighted: Hyperintense

Diffusion-Weighted Imaging (DWI): Marked hyperintensity, which is diagnostically specific

Computed Tomography (CT): May reveal a hypodense, non-enhancing lesion; occasionally shows calcification.

Neurological Examination: To assess cranial nerve function, motor strength, coordination, and reflexes depending on tumor location.

Treatment Strategies

1. Microsurgical Resection

Microsurgical excision is the primary and most effective treatment.

The aim is maximal safe resection, which may be subtotal due to the tumor’s adherence to delicate neurovascular structures.

Tools like neuronavigation, intraoperative neuromonitoring, and microsurgical techniques help minimize functional damage.

Total removal often offers long-term relief, but subtotal resection carries a risk of recurrence.

2. Stereotactic Radiosurgery (SRS)

Considered for residual or recurrent lesions not amenable to re-operation.

While epidermoid tumors are generally radioresistant, SRS might help control tumor growth in select cases.

3. Postoperative Monitoring

MRI follow-up is recommended to detect recurrence, especially after partial removal.

Clinical reassessment for cranial nerve recovery or emerging symptoms is also essential.

Prognosis and Outcomes

Long-term outcomes are generally favorable, particularly when complete or near-complete tumor excision is achieved. These tumors are non-malignant and do not typically undergo cancerous transformation, though malignant degeneration (epidermoid carcinoma) has been reported in extremely rare instances. Recurrence is mostly due to residual epithelial cells left behind after incomplete resection.

Key Points

Epidermoid tumors are congenital, benign lesions that can cause serious neurological symptoms depending on their location. Microsurgical removal remains the gold standard treatment. Close long-term monitoring is essential due to potential recurrence.