Clinical manifestation, management and prognosis of clear cell meningioma: an evidence-based review.

Clear cell meningioma (CCM) is an uncommon histologic subtype of meningioma classified as a WHO grade II tumor and accounting for less than 1% of all meningiomas. Demographically, younger patients are commonly affected without any remarkable gender preference. Moreover, CCM shows a unique anatomical site of involvement. It tends to occur in the cranium than the spine, whereas the basilar skull, posterior fossa and lumbar spine have been the most frequently affected area. Although most cases present as typical the mass effect by the tumor, CCM exhibits characteristic imaging and histologic patterns. Even though surgical resection is the treatment of choice, recurrence-free survival is the biggest challenge and has been attempting to improve by adjuvant therapy. There is still debate about its management, outcome and factors defining it. Herein, we aimed to summarize natural history, radiographic characteristics, histological features, treatment strategies to guide the best possible individualized care for the most favorable outcome.