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

Clinical, Histopathologic and Genetic Features of Rhabdoid Meningiomas

“…From the prognostic point of view, previous studies suggest more aggressive behavior from RM that is associated with a high rate of local recurrence and even distant metastases [47][48][49][50][51], which is confirmed in our cohort. This is in line with previous observations which show that 40% of patients develop tumor recurrences [5,8,[19][20][21]52,53], which is a relatively large fraction of our RM developed tumor recurrence in association with an increased rate of deaths. Despite this, it should be noted that the degree of surgical resection of the tumor might play a role in determining the variable frequency of relapses reported among RM cases.…”

“…Several reports indicated that RM are usually WHO grade 3 meningiomas associated with an adverse prognosis [ 20 , 21 , 34 , 38 , 39 ], although controversial results exist in the literature in this regard [ 8 , 11 , 19 ]. Such discrepancies might be due, at least in part, to the underlying genetic heterogeneity of this rare subtype of meningiomas.…”

“…Based on their CNA profile, RM could be classified into two well-defined genetic profiles (Figure 2c) consisting of (1) RM tumors presenting with multiple chromosomal losses (8/24 tumors, 33%) with a median of 4 (range: 2-6) altered chromosomes in the absence of chromosome gains and (2) RM in which chromosomal losses and gains that involved several chromosomal regions coexisted (with a median of 10 affected chromosomes; range: 3-19 chromosomes) (Figure 2c). Several reports indicated that RM are usually WHO grade 3 meningiomas associated with an adverse prognosis [20,21,34,38,39], although controversial results exist in the literature in this regard [8,11,19]. Such discrepancies might be due, at least in part, to the underlying genetic heterogeneity of this rare subtype of meningiomas.…”

“…From a prognostic point of view, not every RM displays an aggressive clinical course. Thus, despite a tendency toward local recurrence [ 7 , 10 , 17 , 18 , 19 ], and because even distant metastasis in RM has been reported previously [ 5 , 10 , 20 , 21 ], the specific mechanisms leading to tumor relapse still remain to be elucidated. This is due at least in part to the inclusion in previously reported series of RM cases of patients whom could not always attain complete tumor resection and of cases with insufficiently long follow-up times required to reach definitive conclusions about patient outcomes.…”

Paediatric Rhabdoid Meningioma: Clinical and Therapeutic Features Findings – Case Series of 3 Patients

“…The outcome of the reported juvenile RMs seems to be better than their adult counterparts, in which mortality rate was of 49.2% after a mean follow-up of 1 year [12]. Prognosis seems to be related to the quality of surgical resection, the delay between surgery and radiotherapy, and the proliferative activity on histological study [3, 29, 30].…”