• Tae Hoon Roh, Yong Sook Park, Yong Gou Park, Se Hoon Kim, and Jong Hee Chang.
• Yonsei University Graduate School.
• Medicine (Baltimore). 2017 Dec 1; 96 (51): e9423.

RationaleMost of the intracranial epidermoid cysts are benign, but malignant lesions are occasionally reported. These lesions appear as squamous cell carcinoma and carry a dismal prognosis. Here, we report a case of a primary intracranial squamous cell carcinoma arising in a cerebellopontine epidermoid cyst. The relevant literatures were also reviewed.Patient ConcernsA 53-year-old woman presented with dizziness and diplopia 9 months in duration. Magnetic resonance imaging revealed an epidermoid cyst in the left cerebellopontine angle and prepontine region with a focal enhancing lesion on T1-weighted gadolinium-enhanced images.DiagnosesHistopathologic diagnosis revealed squamous cell carcinoma on a background of epidermoid cyst. Imaging studies excluded metastases.InterventionsThe tumor was removed subtotally through a lateral suboccipital approach. The patient received intensity modulated radiation therapy (6720 cGy total) postoperatively.OutcomesThe patient was free from recurrence of the tumor until 3 years after surgery, at which point she was lost to follow-up. The patient died 4 years after the surgery.LessonsThe epidermoid cyst may occasionally become malignant. Finding an area of enhancement through preoperative magnetic resonance imaging can help to make a correct diagnosis. Based on the review of previous reports, surgical removal followed by radiotherapy shows the best result to treat malignant epidermoid cysts.Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

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