Malignant transformation of posterior fossa epidermoid cyst

The authors report the case of a 45-year-old man who presented with a short duration of a painful ophthalmoparesis. Initial magnetic resonance imaging revealed an extraaxial petroclival mass characteristic of an epidermoid cyst, with the exception of a contiguous contrast-enhancing lobule. A subtotal resection was performed with the histopathological diagnosis revealing malignant transformation of an epidermoid cyst. Despite aggressive postoperative adjuvant therapy, the patient developed leptomeningeal metastasis and died shortly thereafter. The presence of contrast enhancement at the site of an epidermoid cyst combined with an acute, progressive neurological deficit should alert the treating physician to the possibility of a malignant transformation. When transformation does occur, the clinical and radiological course is quite aggressive as compared with the indolent growth of epidermoid cysts. Treatment options include surgery with adjuvant chemotherapy or radiotherapy. We review the pertinent features of this case along with the relevant literature regarding primary intracranial squamous cell carcinomas.