Prognostic factors and long-term outcomes of primary intracranial rhabdoid meningioma: A systematic review

Primary intracranial Rhabdoid meningioma (PIRM) is an uncommon subtype of WHO grade III meningioma. Given its rarity, its risk factors and management strategies are still unclear. Therefore, we aimed to assess the risk factors and outcomes for patients with PIRM and proposed an appropriate treatment. Ovid, Medline, Embase, Pubmed, Web of Science and Cochrane database were used to search for articles published between January 1998 and October 2019. Search terms combined intracranial, brain, and cerebral with rhabdoid meningioma or WHO grade III meningioma. The entire cohort included 27 males (51.9 %) and 25 females (48.1 %) with an age ranging from 2 to 77 years (median 44 years). The size of tumor ranged from 1.3 to 7.4 cm (mean 4.3 cm). The Ki-67 proliferation index ranged from 1 % to 90 % (mean 15 %). In the whole cohort, gross total resection (GTR) and non-GTR were achieved in 63.5 % (33 cases) and 36.5 % (19 cases) patients, respectively. Twenty-five patients (48.1 %) had the postoperative radiotherapy, and 5 patients (9.6 %) had postoperative chemotherapy. Nineteen patients (39.6 %) developed recurrences, 4 patients (7.7 %) developed distant metastasizes, and 13 patients (25.0 %) died. GTR was associated with favorable overall survival (p = 0.008). The 1-, 3-, and 5-year progression-free survival rates were 84.6 %, 59.4 %, and 49.6 %, respectively; and the 1-, 3 and 5year overall survival rates in the entire group were 91.4 %, 83.5 % and 68.9 %, respectively. GTR is recommend as the initial treatment option for PIRMs, contributing to acute histological diagnosis and prolonging long-term survival.