Background: Primary intracranial squamous cell carcinoma (SCC) is a rare neoplasm associated with malignant transformation of benign epidermoid or dermoid cysts. The optimal treatment and prognosis of this rare disease are unclear.
Methods: A comprehensive literature review identified all reports relevant to clinical presentation, treatment, and outcome of primary intracranial SCC. All available data were extracted from the included literature. Two patients diagnosed with malignant transformation of an epidermoid cyst in our institute were also included in the study. Survival analysis was conducted to determine the factors affecting patient outcomes.
Results: A total of 62 cases were identified and selected for the present study. The median survival regardless of treatment was 12.8 months. Median survival for patients treated with surgery alone and surgery with radiotherapy was 5 months and 35 months, respectively (P = 0.037). Patients who underwent gross total resection showed relatively increased survival compared with those who underwent subtotal resection (median, 48 months vs. 25 months; P = 0.067). Patients with leptomeningeal carcinomatosis had a significantly decreased median survival of 10 months, as opposed to 41 months (P = 0.005).
Conclusions: Primary intracranial SCC shows poor prognosis, with controversial management. The results of this study indicate that complete resection of tumor when possible, followed by radiotherapy, is the optimal treatment for improving patient outcome.
Keywords: Epidermoid cyst; Intracranial; Malignant transformation; Outcome; Squamous cell carcinoma; Treatment.
Copyright © 2018. Published by Elsevier Inc.