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Central nervous system clear cell meningioma: a systematic literature review

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Abstract

Clear cell meningiomas are a rare histological subtype of World Health Organization (WHO) grade II meningioma. Despite its relatively low frequency, clear cell meningioma has attracted considerable attention because of its unique pathological characteristics, clinical behavior, and challenging management considerations. The purpose of our systematic review is to provide clinicians with a better understanding of this rare disease. PubMed was searched for articles in the English language published from 1988 to 2023 June. The keywords were as follows: “clear cell meningioma,” “clear cell” and “meningioma.” We analyzed clinical manifestations, radiological manifestations, pathological features, comprehensive treatment strategies, and prognosis to determine the factors influencing recurrence-free survival (RFS). Recurrence-free survival curves of related factors were calculated by the Kaplan‒Meier method. The log-rank test and Cox univariate analysis were adopted to assess the intergroup differences and seek significant factors influencing prognosis and recurrence. Fifty-seven papers met the eligibility criteria, including 207 cases of clear cell meningioma (CCM), which were confirmed by postoperative pathology. The fifty-seven articles involved 84 (40.6%) males and 123 (59.4%) females. The average age at diagnosis was 27.9 years (range, 14 months to 84 years). Among the symptoms observed, headache, neurologic deficit, and hearing loss were the most commonly reported clinical manifestations. Most tumors (47.8%) were located in the skull base region. Most tumors showed significant enhancement, and homogeneous enhancement was more common. A total of 152 (74.1%) patients underwent gross total resection (GTR), and 53 (25.9%) patients underwent subtotal resection (STR). During the follow-up, the tumor recurred in 80 (39.4%) patients. The log-rank test and the Cox univariate analysis revealed that tumor resection range (GTR vs. STR) and adjuvant treatment (YES vs. NO) were significant predictors of recurrence-free survival (RFS). Clear cell meningioma is a rare type of meningioma with challenging diagnosis and therapy. The prognosis of this disease is different from that of regular meningiomas. Recurrence remains a possibility even after total tumor resection. We found that the surgical resection range and adjuvant treatment affected the recurrence period. This finding provides significant guidance for the treatment of clear cell meningioma.

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Publicly available datasets were analyzed in this study. These data can be found here: https://pubmed.ncbi.nlm.nih.gov/.

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  1. Department of Neurosurgery, First Hospital of Jilin University, 71 Xinmin Street, Changchun, 130021, Jilin, People’s Republic of China

    Qi Liang, Pengfei Ge, Yanhua Liu, Xiaoxi Zhu, Shan Lu, Chengliang Pan, Zhilin Ji, Qingxuan Wang & Yubo Wang

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Qi Liang and Yubo Wang made substantial contributions to the conception and the acquisition. Qi Liang, Yanhua Liu, Xiaoxi Zhu, and Shan Lu made contributions to analysis and interpretation of data. Qi Liang, Chengliang Pan, Zhilin Ji, and Qingxuan Wang prepared Figs. 1 and 2 and Table 1, 2 and 3. Qi Liang wrote the main manuscript text. Qi Liang, Pengfei Ge, and Yubo Wang drafted the work and revised it critically for important intellectual content, approved the version to be published, and agree to be accountable for all aspects of the work.

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Liang, Q., Ge, P., Liu, Y. et al. Central nervous system clear cell meningioma: a systematic literature review. Neurosurg Rev 47, 35 (2024). https://doi.org/10.1007/s10143-023-02251-6

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