Intrinsic brainstem epidermoid cyst

Case report and review of the literature

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✓ Brainstem epidermoid cysts are rare lesions, with only 18 reported cases in the literature and only five purely intrinsic epidermoid cysts within this group. The authors present the case of a 3-year-old girl with a history of chronic headaches, progressive diplopia, and relapsing and remitting mild right hemiparesis who was found to harbor an intrinsic brainstem epidermoid cyst at the pontomedullary junction. Initial working diagnoses included intrinsic brainstem astrocytoma and cavernoma. After tumor enlargement and progressive symptoms, a diffusion-weighted (DW) magnetic resonance (MR) imaging sequence was performed and a definitive diagnosis of an intrinsic brainstem epidermoid cyst was made in the patient. The patient underwent a suboccipital craniotomy and complete resection of the cyst with the aid of intraoperative neurophysiological monitoring. Three years after the operation, the patient is neurologically intact and no evidence of tumor recurrence has been found. The rarity of brainstem epidermoid cysts can make their diagnosis difficult; thus a DW MR imaging sequence of the brain is a useful diagnostic modality. Intrinsic brainstem epidermoid cysts can be removed safely, in a manner similar to that used for the surgical treatment of focal tumors.

Abbreviations used in this paper: CPA = cerebellopontine angle; DW = diffusion-weighted; MR = magnetic resonance.

Article Information

Address reprint requests to: George I. Jallo, M.D., Division of Pediatric Neurosurgery, The Johns Hopkins University Hospital, 600 North Wolfe Street, Harvey 811, Baltimore, Maryland 21287. email: gjallo1@jhmi.edu.

© AANS, except where prohibited by US copyright law.

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    Images from the early workup when the patient was 17 months of age. Left: A T1-weighted sagittal MR image with no contrast enhancement demonstrating a small lesion at the anterior pontomedullary junction. Center: Axial T1-weighted Gd-enhanced image demonstrating intrinsic location and slight enhancement. Right: A T2-weighted sequence demonstrating the hyperintense signal within the brainstem. There is no lesion visible within the subarachnoid space.

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    Immediately preoperative MR images (age 35 months). Upper Left: Sagittal T1-weighted sequence demonstrating significant enlargement of the tumor within the pontomedullary brainstem. Upper Right: Axial T1-weighted image (with Gd contrast) demonstrating minimal to no enhancement in this large tumor. Lower Left: A T2-weighted axial image demonstrating a possible cystic lesion situated within the brainstem. Lower Right: An axial DW image of the brainstem confirming the clinical suspicion of an epidermoid cyst.

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    Two-year postoperative MR images. Upper Left: A T1-weighted sagittal MR image demonstrating the lack of residual tumor within the brainstem. Upper Right: A T1-weighted axial image in which no evidence of recurrent or residual disease was found. Lower Left: A T2-weighted axial image demonstrating a normal signal within the brainstem. Lower Right: Axial DW image confirming the gross-total resection.

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