Table 1.
Systematic review of the literature pertaining to the natural history, clinical features, and treatment strategies of cortical ependymomas.
| Author (Year) | Age/Sex | Symptoms | Gene fusion | WHO grade | Location | Cystic | Treatment | Recurrence | OS (months) | PFS (months) | Last FU (months) | Outcome | Ref |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Saito et al. (1999) | 63F | Seizure | NR | 2 | L parietal | No | GTR + RT | No | NA | NA | 14 | Alive | 7 |
| Fujimoto et al. (1999) | 13M | Hyperesthesia | NR | 2 | L frontoparietal | Yes | GTR | NR | NR | NR | NR | NR | 8 |
| Sato et al. (2000) | 41F | Weakness | NR | 3 | L frontoparietal | Yes | GTR + RT | No | NA | NA | 2 | Alive | 9 |
| Takeshima et al. (2002) | 70F | Incidental | NR | 3 | R frontal | Yes | GTR | No | NA | NA | 14 | Alive | 10 |
| Lehman et al. (2003) | 10M | Seizure | NR | 2 | R frontal | Yes | GTR | No | NA | NA | 19 | Alive | 11 |
| Roncaroli et al. (2005) | 52M | Seizure | NR | 2 | L frontal | No | GTR + RT | No | NA | NA | 130 | Alive | 12 |
| 34M | Seizure | NR | 2 | L temporal | No | GTR | No | NA | NA | 100 | Alive | ||
| 7F | Seizure | NR | 2 | R parietal | No | GTR | No | NA | NA | 48 | Alive | ||
| Miyazawa et al. (2007) | 33M | Aphasia | NR | 3 | L parietal | No | GTR + RT + ChT | Yes | NA | 6 | 7 | Alive | 13 |
| Ghani et al. (2008) | 4M | Seizure | NR | 2 | L frontoparietal | Yes | GTR +RT | Yes | NA | 10 | NR | Alive | 14 |
| Lehman (2008) | 1F | Seizure | NR | 3 | R frontal | No | GTR | No | NA | NA | 48 | Alive | 15 |
| Grajkowska et al. (2009) | 11F | Headache | NR | 2 | L frontoparietal | Yes | GTR + RT | No | NA | NA | 72 | Alive | 16 |
| Yadav et al. (2009) | 15M | Headache | NR | 2 | L frontal | No | STR | Stable | NA | NA | 20 | Alive | 17 |
| Niazi et al. (2009) | 36F | Seizure | NR | 3 | R frontal | Yes | GTR + RT | No | NA | NA | 29 | Alive | 18 |
| 18M | Seizure | NR | 3 | R frontoparietal | Yes | GTR + RT + ChT | Yes | 14 | 6 | 14 | Deceased | ||
| 28F | Seizure | NR | 2 | L temporoparietal | Yes | GTR | No | NA | NA | 42 | Alive | ||
| Yurt et al. (2010) | 11M | Seizure | NR | 2 | L frontal | Yes | GTR | No | NA | NA | 12 | Alive | 19 |
| Lee et al. (2011) | 2M | Seizure | NR | 2 | R frontoparietal | No | GTR | No | NA | NA | 12 | Alive | 20 |
| Van Gompel et al. (2011) | 32F | Incidental | NR | 2 | L parietal | Yes | GTR | No | NA | NA | 59 | Alive | 21 |
| 43F | Seizure | NR | 2 | L frontal, insular | Yes | STR + RT | Stable | NA | NA | 60 | Alive | ||
| 12M | Seizure | NR | 3 | R parietal | Yes | GTR + RT | Yes | NA | NR | 101 | Alive | ||
| 40F | Seizure | NR | 2 | R frontal | Yes | GTR | No | NA | NA | 131 | Alive | ||
| 25M | Seizure | NR | 3 | R frontal | Yes | GTR + RT | No | NA | NA | 80 | Alive | ||
| 26M | Seizure | NR | 2 | R occipital | Yes | GTR | No | NA | NA | 6 | Alive | ||
| 59F | Incidental | NR | 2 | L parietal | No | GTR | No | NA | NA | 31 | Alive | ||
| 59M | Seizure | NR | 3 | R frontal | No | GTR + RT | Yes | NA | NR | 47 | Alive | ||
| 25F | Seizure | NR | 2 | L frontal | Yes | GTR | No | NA | NA | 39 | Alive | ||
| Davis et al. (2011) | 22F | Headache | NR | 3 | R frontotemporal | Yes | GTR + RT | Yes | NA | 20 | 55 | NR | 22 |
| Romero et al. (2012) | 23M | Seizure | NR | 3 | L frontal | Yes | GTR + RT | No | NA | NA | 60 | Alive | 23 |
| Ng et al. (2012) | 51F | Incidental | NR | 3 | Bifrontal | Yes | GTR + RT | Yes | NA | 4 | 8 | Alive | 24 |
| Nakamizo et al. (2012) | 20F | Seizure | NR | 2 | L frontoparietal | No | GTR | No | NA | NA | 16 | Alive | 25 |
| Ohla et al. (2012) | 29M | Headache | NR | 3 | L parietal | No | STR + RT | Yes | 15 | 14 | 15 | Deceased | 26 |
| Rigante et al. (2013) | 14M | Seizure | NR | 2 | R temporoparietal | Yes | GTR | No | NA | NA | 12 | Alive | 27 |
| Hiniker et al. (2013) | 19F | Seizure | NR | NR | L frontotemporal | Yes | STR | Stable | NA | NA | 4 | Alive | 28 |
| Elsharkawy et al. (2013) | 25M | Seizure | NR | 3 | R frontal | No | GTR | No | NA | NA | 6 | Alive | 29 |
| Kambe et al. (2014) | 2M | Seizure | NR | 2 | R parietal | NR | GTR | No | NA | NA | NR | NR | 30 |
| Liu et al. (2014) | 24M | Seizure | NR | 2 | R frontal | No | GTR | No | NA | NA | 24 | Alive | 31 |
| 30M | Seizure | NR | 3 | R parietal | No | GTR + RT + ChT | Yes | NA | NR | 264 | Alive | ||
| 50M | Seizure | NR | 3 | L frontal | Yes | GTR + RT +ChT | Yes | 72 | NR | 72 | Deceased | ||
| 50F | Seizure | NR | 3 | L frontal | No | GTR | Yes | NA | NR | 150 | Alive | ||
| 2M | Hemiparesis | NR | 3 | L frontal | No | GTR | Yes | NA | NR | 48 | Alive | ||
| 47F | Headache | NR | 3 | R frontal | Yes | STR | NR | <1 | NR | <1 | *Deceased Operative complications | ||
| 54F | Headache | NR | 3 | R parietal | Yes | GTR | NR | 48 | NR | 48 | Deceased | ||
| 52F | Confusion | NR | 3 | L frontal | No | GTR | Yes | NA | NR | 36 | Alive | ||
| 15F | Hemiparesis | NR | 3 | L temporal | Yes | GTR + RT + ChT | No | NA | NA | 11 | Alive | ||
| 20M | Seizure | NR | 2 | L temporal | No | GTR | No | NA | NA | 6 | Alive | ||
| 63F | Headache | NR | 3 | L parietal | Yes | GTR + RT | NR | 4 | NR | 4 | Deceased | ||
| Zhang et al. (2014) | 1.5M | Seizure | NR | 3 | R frontoparietal | Yes | GTR | No | NA | NA | 6 | Alive | 32 |
| Tailor et al. (2015) | 27F | Seizure | NR | 2 | L parietal | Yes | GTR | NR | NA | NA | NR | NR | 33 |
| Yamasaki et al. (2015) | 10M | Seizure | NR | 2 | R parietal | N/A | GTR | No | NA | NA | 12 | Alive | 34 |
| Bijwe et al. (2015) | 14F | Headache | NR | 2 | R frontal | Yes | GTR | No | NA | NA | 6 | Alive | 35 |
| Mohaghegh et al. (2015) | 17M | Weakness | NR | 3 | L parietooccipital | Yes | GTR | NR | NA | NA | NR | NR | 36 |
| Kharosekar et al. (2018) | 11F | Headache | NR | 3 | R frontoparietal | Yes | GTR | NR | NA | NA | NR | NR | 37 |
| Wang et al. (2018) | 6M | Seizure | NR | 2 | R frontal | No | GTR | No | NA | NA | 23 | Alive | 38 |
| 13F | Seizure | NR | 2 | R parietal | Yes | GTR | No | NA | NA | 48 | Alive | ||
| 46M | Seizure | NR | 3 | L parietal | No | GTR | No | NA | NA | 46 | Alive | ||
| 5F | Headache | NR | 2 | L frontal | Yes | GTR | No | NA | NA | 70 | Alive | ||
| 74M | Headache | NR | 3 | R frontal | Yes | GTR | Yes | 20 | 11 | 20 | Deceased | ||
| 4F | Seizure | NR | 2 | R frontal | Yes | GTR | No | NA | NA | 71 | Alive | ||
| 49M | Seizure | NR | 2 | L temporal | No | GTR | No | NA | NA | 88 | Alive | ||
| 9M | Seizure | NR | 3 | R frontal | Yes | STR + RT | Yes | NA | NR | 41 | Alive | ||
| 31F | Seizure | NR | 2 | R parietal | No | GTR | No | NA | NA | 67 | Alive | ||
| 18M | Seizure | NR | 3 | L temporal | No | GTR + RT | No | NA | NA | 39 | Alive | ||
| 53F | Seizure | NR | 2 | R temporal | No | GTR | No | NA | NA | 51 | Alive | ||
| 51M | Seizure | NR | 2 | L parietal | No | GTR | No | NA | NA | 75 | Alive | ||
| 45F | Seizure | NR | 2 | R parietal | Yes | GTR | No | NA | NA | 35 | Alive | ||
| Beniwal et al. (2018) | 3F | Dyskinesia | NR | 3 | L frontal | NR | GTR | No | NA | NA | 12 | Alive | 39 |
| Sun et al. (2018) | 19M | Numbness | NR | 2 | L parietal | No | GTR | No | NA | NA | 8 | Alive | 40 |
| 26F | Headache | NR | 3 | R frontoparietal | Yes | GTR + RT | No | NA | NA | 15 | Alive | ||
| 24M | Seizure | NR | 2 | L temporal | No | GTR | No | NA | NA | 30 | Alive | ||
| 6M | Headache | NR | 3 | L frontal | No | STR + RT | Yes | 25 | 18 | 25 | Deceased | ||
| 30F | Headache | NR | 2 | L occipital | No | GTR | No | NA | NA | 25 | Alive | ||
| 22M | Headache | NR | 3 | R temporal | No | GTR + RT | Yes | 22 | 15 | 22 | Deceased | ||
| 31M | Headache | NR | 3 | R frontal | Yes | STR + RT | No | NA | NA | 28 | Alive | ||
| 18F | Dizziness | NR | 2 | L temporal | No | GTR | No | NA | NA | 32 | Alive | ||
| 3F | Seizure | NR | 3 | R frontal | Yes | GTR + RT | No | NA | NA | 15 | Alive | ||
| 22M | Seizure | NR | 2 | R temporal | Yes | GTR | No | NA | NA | 14 | Alive | ||
| 11F | Vomiting | NR | 2 | L parietooccipital | Yes | GTR | No | NA | NA | 8 | Alive | ||
| 48M | Headache | NR | 2 | L frontal | No | GTR | No | NA | NA | 39 | Alive | ||
| 35F | Seizure | NR | 3 | R parietal | Yes | GTR + RT | Yes | NA | 18 | 21 | Alive | ||
| Matsumoto et al. (2019) | 9M | Headache | NR | 3 | R frontal | Yes | STR + RT | Yes | NA | 32 | 347 | Alive | 41 |
| 16F | Vomiting | NR | 3 | L frontal | Yes | GTR + RT | Yes | NA | 19 | 23 | NR | ||
| 4M | Headache | NR | 3 | L frontal | Yes | GTR + RT | Yes | NA | 16 | 74 | Alive | ||
| 6M | Hemiparesis | RELA fusion | 2 | L parietal | Yes | STR | Yes | NA | 25 | 187 | Alive | ||
| 22M | Hemianopsia | RELA fusion | 3 | L parietal | Yes | GTR + RT | No | NA | NA | 104 | Alive | ||
| 3M | Seizure | RELA fusion | 3 | R frontal | Yes | GTR + RT | No | NA | NA | 136 | Alive | ||
| 24M | Seizure | RELA fusion | 3 | L parietal | Yes | GTR + RT | No | NA | NA | 74 | Alive | ||
| 1F | Seizure | RELA fusion | 3 | L parietal | Yes | GTR | No | NA | NA | 10 | Alive | ||
| *Khatri et al. (2019) | 32M | Headache | NR | 3 | NR | NR | GTR + RT | No | NA | NA | 101 | Alive | 42 |
| 11M | Headache | NR | 3 | NR | NR | GTR + RT | No | NA | NA | 101 | Alive | ||
| 12M | Headache | NR | 3 | NR | NR | GTR + RT | Yes | 53 | NR | 53 | Deceased | ||
| 10M | Seizure | NR | 3 | NR | NR | STR + RT | Yes | NA | NR | 88 | Alive | ||
| 5M | Headache | NR | 2 | NR | NR | GTR | No | NA | NA | 48 | Alive | ||
| 12F | Headache | NR | 3 | NR | NR | STR + RT | No | NA | NA | 47 | Alive | ||
| 16M | Seizure | NR | 2 | NR | NR | GTR | No | NA | NA | 43 | Alive | ||
| 14F | Headache | NR | 3 | NR | NR | GTR + RT | No | NA | NA | 26 | Alive | ||
| 46F | Headache | NR | 2 | NR | NR | GTR | No | NA | NA | 16 | Alive | ||
| 21F | Headache | NR | 3 | NR | NR | GTR + RT | Yes | 11 | NR | 11 | Deceased | ||
| 14F | Headache | NR | 2 | NR | NR | STR | No | 8 | NA | 8 | Deceased | ||
| 8M | Headache | NR | 2 | NR | NR | GTR + RT | Yes | NA | NR | 40 | Alive | ||
| 22M | Headache | NR | 2 | NR | NR | STR + RT | No | NA | NA | 27 | Alive | ||
| 23M | Headache | NR | 2 | NR | NR | STR + RT | No | NA | NA | 20 | Alive | ||
| 22M | Headache | NR | 3 | NR | NR | GTR + RT | No | NA | NA | 14 | Alive | ||
| 14F | Headache | NR | 2 | NR | NR | STR + RT | No | NA | NA | 11 | Alive | ||
| 42M | Seizure | NR | 2 | NR | NR | STR + RT | No | NA | NA | 7 | Alive | ||
| 19M | Headache | NR | 3 | NR | NR | GTR + RT | No | NA | NA | 7 | Alive | ||
| Wang et al. (2020) | 26F | Seizure | NR | 3 | R frontal | Yes | STR | Yes | 43 | NR | 43 | Deceased | 43 |
| 48M | Headache | NR | 2 | L temporal | Yes | STR + RT | Yes | NA | NR | 94 | Alive | ||
| 50M | Headache | RELA fusion | 2 | R frontal | No | GTR | No | NA | NA | 24 | Alive | ||
| 5F | Seizure | NR | 2 | R frontal | No | GTR | No | NA | NA | 24 | Alive | ||
| 5M | Headache | NR | 3 | L frontotemporal | Yes | GTR + RT | No | NA | NA | 50 | Alive | ||
| 54F | Dizziness | No | 3 | R temporal | Yes | STR + RT | Yes | 26 | NR | 26 | Deceased | ||
| 8F | Headache | NR | 3 | R temporooccipital | Yes | STR + RT | Yes | NA | NR | 21 | Alive | ||
| 37M | Headache | NR | 2 | R temporal | No | STR + RT | Yes | NA | NR | 36 | Alive | ||
| 22F | Headache | NR | 2 | R temporal | No | STR + RT | Yes | 29 | NR | 29 | Deceased | ||
| 58M | Seizure | NR | 2 | R temporal | Yes | GTR | No | NA | NA | 60 | Alive | ||
| 17M | Headache | NR | 2 | R frontotemporal | Yes | STR + RT | No | NA | NA | 48 | Alive | ||
| 2F | Headache | NR | 3 | R frontal | Yes | GTR + RT | No | NA | NA | 36 | Alive | ||
| 4M | Headache | NR | 2 | R occipital | Yes | GTR | No | NA | NA | 36 | Alive | ||
| 6F | Hemiparesis | NR | 2 | R frontoparietal | Yes | GTR | No | NA | NA | 30 | Alive | ||
| 5M | Seizure | RELA fusion | 2 | R frontal | No | GTR | No | NA | NA | 29 | Alive | ||
| 7M | Headache | NR | 2 | R occipital | No | GTR | No | NA | NA | 9 | Alive | ||
| 11F | Headache | NR | 2 | R temporal | Yes | GTR | No | NA | NA | 36 | Alive | ||
| 6F | Seizure | RELA fusion | 2 | L temporooccipital | No | GTR | No | NA | NA | 18 | Alive | ||
| 11M | Seizure | RELA fusion | 3 | L temporal | No | GTR + RT | No | NA | NA | 18 | Alive | ||
| 13F | Headache | NR | 3 | R parietal | Yes | STR + RT | No | NA | NA | 48 | Alive | ||
| 2M | Hemiparesis | NR | 3 | L frontoparietal | Yes | STR | Yes | 24 | NR | 24 | Deceased | ||
| 19F | Seizure | NR | 2 | R temporooccipital | Yes | GTR | No | NA | NA | 27 | Alive | ||
| 4M | Headache | RELA fusion | 3 | R parietal | No | GTR + RT | No | NA | NA | 36 | Alive | ||
| 17M | Seizure | RELA fusion | 2 | R temporooccipital | Yes | GTR | No | NA | NA | 8 | Alive | ||
| 22M | Seizure | RELA fusion | 3 | L parietal | No | GTR + RT | No | NA | NA | 6 | Alive | ||
| 4M | Headache | NR | 2 | R temporal | No | GTR | No | NA | NA | 65 | Alive | ||
| 5M | Headache | NR | 3 | R frontal | No | GTR + RT | Yes | NA | NR | 72 | Alive | ||
| 14M | Hemiparesis | NR | 2 | R frontotemporal | Yes | GTR | No | NA | NA | 24 | Alive | ||
| 11M | Hemiparesis | RELA fusion | 3 | R frontoparietal | No | STR + RT | Yes | NA | NR | 7 | Alive | ||
| 0.75M | Vomiting | RELA fusion | 3 | R frontal | Yes | GTR | No | NA | NA | 5 | Alive | ||
| Senthilvelan et al. (2020) | 22M | Seizure | NR | 2 | L frontal | Yes | GTR | NR | NR | NR | NR | NR | 44 |
| Sallam et al. (2020) | 9M | Facial droop | RELA fusion | 2 | L frontal | Yes | GTR | Yes | NA | 5 | 5 | Alive | 45 |
| Safavi et al. (2021) | 2M | Headache | NR | NR | L frontal | Yes | GTR | NR | NR | NR | NR | NR | 46 |
| Lee et al. (2021) | 25F | Seizure | RELA fusion | 2 | R frontoparietal | No | GTR | No | NA | NA | 10 | Alive | 47 |
| Wang et al. (2021) | 6F | Seizure | RELA fusion | 2 | L temporooccipital | No | GTR | No | NA | NA | 18 | Alive | 48 |
| 11M | Seizure | RELA fusion | 3 | L temporal | No | GTR + RT | No | NA | NA | 18 | Alive | ||
| 13F | Headache | NR | 3 | R parietal | Yes | STR + RT | No | NA | NA | 48 | Alive | ||
| 2M | Weakness | NR | 3 | L frontoparietal | Yes | STR | Yes | 24 | 20 | 24 | Deceased | ||
| 6F | Weakness | NR | 2 | R frontoparietal | Yes | GTR | No | NA | NA | 30 | Alive | ||
| 5M | Seizure | RELA fusion | 2 | R frontal | No | GTR | No | NA | NA | 29 | Alive | ||
| 7M | Headache | NR | 2 | R occipital | No | GTR | No | NA | NA | 9 | Alive | ||
| 11F | Headache | NR | 2 | R temporal | Yes | GTR | No | NA | NA | 36 | Alive | ||
| 4M | Headache | NR | 2 | R temporal | No | GTR | No | NA | NA | 65 | Alive | ||
| 5M | Headache | NR | 3 | R frontal | No | GTR + RT | Yes | NA | 24 | 72 | Alive | ||
| 14M | Weakness | NR | 2 | R frontotemporal | Yes | GTR | No | NA | NA | 24 | Alive | ||
| 11M | Weakness | RELA fusion | 3 | R frontoparietal | No | STR + RT | Yes | NA | 7 | 7 | Alive | ||
| 0.75M | Vomiting | RELA fusion | 3 | R frontal | Yes | GTR | No | NA | NA | 5 | Alive | ||
| Current case | 58F | Aphasia | No | 2 | L insula | Yes | STR + RT | No | NA | NA | 15 | Alive |
Abbreviations: WHO: World Health Organization; OS: overall survival; PFS: progression-free survival: FU: follow up; F: female; NR: not reported; L: left: GTR: gross total resection; RT: radiotherapy; NA: not available; M: male; R: right; ChT: chemotherapy; STR: subtotal resection.
*Overall, the frontal lobe accounted for nine cases, parietooccipital lobe for four cases, frontotemporoparietal region for three cases, and temporal lobe for two cases. Fourteen patients had a cystic appearing tumor.