Background: Malignant progression of intracranial dermoid cysts into squamous cell carcinoma is extremely rare with only three reports published so far. Intracranial dermoid cysts are uncommon benign tumors lined by stratified squamous epithelium of embryonic ectodermal origin.
Observations: Here, the authors present the case of a 64-year-old female with a recurrent temporal dermoid cyst. After surgery for the recurrent dermoid cyst, once in the early 1990s and another 16 years later, the patient presented with headache and nausea due to hydrocephalus. After implantation of a ventriculoperitoneal shunt, she deteriorated rapidly and died only 60 days after admission. Autopsy revealed malignant transformation of the epithelial lining of the dermoid cyst into a squamous cell carcinoma resulting in neoplastic meningiosis and intraperitoneal tumor spread along a previously implanted ventriculoperitoneal shunt.
Lessons: Malignant transformation should be considered in patients with dermoid cyst who show new leptomeningeal contrast enhancement. In the case of hydrocephalus, alternatives to peritoneal shunting should be considered.
Keywords: dermoid cyst; intracranial tumor; malignant transformation; peritoneal spreading; squamous cell carcinoma; ventriculoperitoneal shunt.