Primary Squamous Cell Carcinomas Arising in Intracranial Epidermoid Cysts: A Series of Nine Cases and Systematic Review

Epidermoid and dermoid cysts are among the most benign intra cranial tumors. Their malignant transformation into squamous cell carcinoma is rare. The authors reviewed the literature. MEDLINE and SCIENCE DIRECT searches, and examination of the references in the selected articles yielded 74 patients, 52 of whom fulfilled Garcia's criteria and were selected for the study. Survival analyses were performed to determine whether survival differences were of statistical significance, and P < 0.05 was considered as significant. Malignant transformation is characterized by a rapid onset of symptoms, recurrence, leptomeningeal carcinomatosis (LC), and tumor enhancement at Computed Tomography Scan or Magnetic Resonance Imaging (87.8 showed this radiological feature). In this review, the SCCs were classified in five groups: (1) Initial malignant transformation of a benign cyst; (2) malignant transformation from a remnant cyst; (3) malignant transformation of a dermoid and epithelial cyst; (4) malignant transformation with leptomeningeal carcinomatosis; (5) other malignancies arising from benign cysts. The median survival was 9 months. Statistics show that LC was of poor prognosis and radiotherapy, although not statistically significant, seems effective against such lesions, with a median survival of 26 months as opposed to 3 months (P=0.077). Although rare, malignant transformation of intracranial epithelial cysts has a poor prognosis and surgery followed by radiotherapy seems to be the best therapeutic modality.

Malignant transformation of benign epidermoid cysts is rare and their prognosis remains poor. A 56-year-old woman presented with left facial hypoesthesia and photophobia in the left eye. She had undergone removal of a benign epidermoid cyst in the cerebellopontine angle 8 years previously. Magnetic resonance imaging of the brain revealed a cystic lesion in the left cerebellopontine angle. The cyst wall was enhanced by gadolinium-DTPA. She underwent removal again and the histopathologic diagnosis was squamous cell carcinoma. Gamma knife radiosurgery was performed on the remnant lesion with a marginal dose of 15 Gy. The tumor shrank rapidly for 2 months after radiosurgery, but recurred 9 months later. She underwent radiosurgery again with a marginal dose of 12 Gy. A gradual increase in the size of the enhanced lesion was seen during the 4-month follow-up period subsequent to the second radiosurgery. Careful observation, employing serial magnetic resonance images, is necessary for incompletely resected epidermoid cysts because intervals before malignant transformation reportedly range from 3 months to 33 years. Newly identified contrast enhancement strongly indicates malignant change in epidermoid cysts. Gamma knife radiosurgery may be useful for short-term control of intracranial squamous cell carcinomas, but long-term effects are presently unknown.

The authors present the case of a woman with a cerebellopontine angle (CPA) epidermoid cyst that degenerated into a squamous cell carcinoma. Malignant degeneration of an epidermoid cyst is an extremely rare occurrence. Malignant transformation must be considered in the differential diagnosis when new contrast enhancement on imaging studies and progressive neurological deficit are seen in a patient harboring an epidermoid cyst. The patient initially presented with a 10-year history of left trigeminal neuralgia, subacute left-sided hearing loss, and with facial weakness of 3 weeks' duration. Initial magnetic resonance (MR) imaging revealed a left CPA mass, consistent with an epidermoid. There was faint contrast enhancement where the tumor was in contact with the lateral brainstem. A subtotal resection was performed. Histopathological findings were consistent with an epidermoid tumor. One year after initial presentation, the patient's neurological deficit had increased, and follow-up MR imaging demonstrated a large contrast-enhancing tumor filling the left CPA and compressing the brainstem. At repeated surgery a squamous cell carcinoma arising from the previous epidermoid was found. The patient was subsequently treated with external-beam radiotherapy and stereotactic radiosurgery. Her tumor stabilized. Three years and 8 months after the patient's initial presentation, a new area of tumor developed at the torcular Herophili. The patient died shortly thereafter. Malignant squamous degeneration is a rare cause of enhancement on MR images, as is progressive neurological deficit in a patient with an epidermoid. The combination of subtotal resection, external-beam radiotherapy, and stereotactic radiosurgery may be useful for local tumor control but the long-term prognosis is guarded.