Table 1. Summary of cases of adult primary intracranial Ewing sarcoma (peripheral primitive neuroectodermal tumor) reported in the literature.
| Author/year | Age (yr)/sex | Initial symptom | Site | Tumor maximal diameter (cm) | Contrast enhancement | Cystic feature | Dural base | Bone involvement | Initial metastasis | Extent of resection | EWSR1-FLI1 fusion | Chemotherapy | Radiotherapy | Follow-up period (months) | Outcome |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Present | 38/F | Tinnitus, hemiparesis | Petrous ridge, right | 6.0 | Y | Y | Y | N | N | PR | NGS (WES) | VAC/IE until 48 weeks | 55 Gy in 25 Fx | 12 | Alive/controlled |
| Geng et al./2023 [3] | 21/F | IICP signs* | Bilateral PL | 4.0 | Y | Y | Y | N | N | STR | NGS | N | 60 Gy in 30 Fx | 6 | Alive/controlled |
| Deshpande et al./2021 [2] | 33/M | IICP signs* | Left TL | NA | NA | NA | NA | NA | NA | Excision | RT-PCR | VAC/IE until 48 weeks | 55.8 Gy in 31 Fx | 18 | Alive/controlled |
| Deshpande et al./2021 [2] | 33/F | Left eye movement impairment, left frontal swelling | Left FL | NA | NA | NA | NA | NA | NA | Excision | FISH | VC until 2 weeks (discontinued due to intolerance) | 54 Gy in 30 Fx | 17 | Alive/recurred after 17 months |
| Jiang et al./2020 [4] | 55/F | Memory impairment | Left FL | 6.5 | Y | Y | Y | N | N | GTR | FISH | N | Y | 18 | Alive |
| Cherif El Asri et al./2018 [5] | 28/M | IICP signs,* left V, VI, VII, VIII, and lower cranial nerves palsies | Left CPA | 5.0 | Y | N | Y | N | N | PR | FISH | VCD/IE x1 | 56 Gy | 3 | Died due to nosocomial pneumopathy |
| Ke et al./2017 [10] | 43/M | Epilepsy | Right PL | NA | Y | NA | Y | N | N | GTR | FISH | VACA/VAIA | 50 Gy | 48 | Alive |
| Kumar et al./2017 [11] | 22/M | Seizure, right hemiparesis | Left FL | 8.8 | Y | N | Y | Y | N | GTR | NA | Y | Y | 7 | Alive/controlled |
| VandenHeuvel et al./2015 [12] | 61/M | Motor dysphasia and left-sided weakness | Right TL | 6.2 | Y | Y | Y | NA | N | GTR | FISH | N | N | NA | NA |
| Salunke et al./2014 [13] | 52/M | IICP sign,* fever, diminution of vision, and ataxia | Posterior fossa | 6.0 | Y | NA | Y | Y | N | NTR | NA | VAC/IE until 16 weeks | 50 Gy | 10 | Died/locally recurred after 10 months |
| Cole et al./2014 [14] | 51/F | Visual disturbance | Left TL + PL | 3.5 | Y | N | Y | N | N | GTR | FISH | VCD/IE x4 VAC/IE x10 | N | 24 | Alive |
| Tanboon et al./2012 [9] | 22/F | Enlarging skull mass in right frontal resion | Right FL | 8.0 | Y | N | Y | Y | N | STR | FISH | N | N | 6 | Died |
| Antonelli et al./2011 [15] | 37/M | Comatose state following IICP sign* | Right FL + TL | NA | Y | Y | Y | N | N | GTR | RT-PCR | N | Y | 5 | Alive/controlled |
| Mellai et al./2010 [16] | 56/F | IICP sign,* left hemiparesis | Right TL | NA | Y | Y | Y | Y | NA | GTR | RT-PCR | N | N | 18 | Alive/controlled |
| Attabib et al./2006 [17] | 48/F | Headache and left maxillary and oculomotor nerve palsies | Lt cavernous sinus | 4.0 | Y | N | N | N | N | STR | RT-PCR | VCD/IE | 54 Gy | 14 | Alive/controlled |
| Mobley et al./2006 [18] | 21/M | IICP sign,* diplopia, left partial homonymous hemianopia | Right OL | 3.5 | Y | N | Y | N | N | PR | Karyotyping | VACA | 54 Gy | 21 | Alive/recurred after 18 months |
| D’Antonio et al./2004 [19] | 50/F | IICP sign* | Right TL | NA | Y | N | Y | NA | N | GTR | FISH | N | N | 12 | Alive |
| Simmons et al./2001 [20] | 67/F | Right facial pain, facial palsy, hearing disturbance, headache | Right CPA | NA | Y | NA | N | N | N | Biopsy | NA | N | Y | 13 | Died/rapid decline following no symptoms progression for 13 months |
| Kalamarides et al./2001 [21] | 34/F | Vertigo, tinnitus, gait disturbance | Left CPA | 1.8 | Y | N | Y | Y | N | STR | NA | N | 55 Gy | 12 | Alive/controlled |
| Papotti et al./1998 [22] | 30/F | Headache, vertigo | Right FL (2 lesions) | 7 and 4 | NA | NA | Y | NA | NA | GTR (misdiagnosed as a meningioma) | RT-PCR | VAC (at 9 years after initial surgery) | 50 Gy (misdiagnosed as a meningioma) | 120 | Died/recurred twice at 7 and 9 years after initial surgery |
*IICP signs included headache, nausea, vomiting, or loss of consciousness. M, male; F, female; IICP, increased intracranial pressure; PL, parietal lobe; FL, frontal lobe; TL, temporal lobe; OL, occipital lobe; CPA, cerebellopontine angle; NA, not applicable (or no information); Y, yes; N, no; GTR, gross total resection; NTR, near total resection; STR, subtotal resection; PR, partial resection; NGS, next generation sequencing; WES, whole exome sequencing; RT-PCR, reverse transcriptase-polymerase chain reaction; FISH, fluorescence in situ hybridization; VAC, vincristine, adriamycin, and cyclophosphamide; IE, ifosfamide and etoposide; VC, vincristine and cyclophosphamide; VCD, vincristine, cyclophosphamide, and doxorubicin; VACA, vincristine, adriamycin, cyclophosphamide, and dactinomycin; VAIA, vincristine, adriamycin, ifosfamide, and dactinomycin; Fx, fractions