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2000
Volume 21, Issue 1
  • ISSN: 1573-4056
  • E-ISSN: 1875-6603
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Abstract

Background

Primary intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) is exceedingly rare and easy to misdiagnose.

Case Presentation

We present a case involving a 23-year-old male who presented with headaches and vomiting. The preoperative brain imaging revealed an irregular mass in the left parietal lobe, initially misdiagnosed as meningioma. However, the surgical specimen was ultimately diagnosed as primary intracranial EWS/pPNET. The patient underwent a total tumor resection, followed by adjuvant chemotherapy and radiotherapy. No recurrence or distant metastasis was observed 18 months after the surgery.

Conclusion

When the imaging features of young patients’ lesions are solid, aggressive, and unevenly enhanced masses, physicians should be aware of the possibility of primary intracranial EWS/pPNET, and if possible, Gross Total Resection (GTR) and intensive chemotherapy and radiotherapy are recommended.

This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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2025-01-01
2025-04-25
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References

  1. HavemanL.M. RanftA. van den BergH. BrosiusK.S. LadensteinR. PaulussenM. JuergensH. DirksenU. MerksJ.H.M. Primary and metastatic intracranial Ewing sarcoma at diagnosis: Retrospective international study and systematic review.Cancers2020126167510.3390/cancers1206167532599807
    [Google Scholar]
  2. MatignonP P NasiD IaccarinoC First case of primary sellar/suprasellar-intraventricular Ewing sarcoma: Case report and review of the literature.World Neurosur.201798869. e1869. e5
    [Google Scholar]
  3. YimJ. LeeW.S. KimS.K. KangH.J. BaeJ. ParkS.H. Intracranial Ewing sarcoma with whole genome study.Childs Nerv. Syst.201935354755210.1007/s00381‑018‑3997‑130406421
    [Google Scholar]
  4. JiangY. ZhaoL. WangY. LiuX. WuX. LiY. Primary intracranial Ewing sarcoma/peripheral primitive neuroectodermal tumor mimicking meningioma: A case report and literature review.Front. Oncol.20201052807310.3389/fonc.2020.52807333123461
    [Google Scholar]
  5. SalunkeP.S. GuptaK. MalikV. KumarN. HenkeL.E. CaiC. ChenW.S. PfeiferJ.D. Primary Ewing’s sarcoma of cranial bones: Analysis of ten patients.Acta Neurochir.201115371477148510.1007/s00701‑011‑1028‑z21516517
    [Google Scholar]
  6. RavinaK. WindermereS.A. ZhaoQ. LernerA. DyerM. UpadhyayU. JhaR.T. Primary intracranial extraosseous Ewing’s sarcoma of the skull base in an elderly adult: Illustrative case.J. Neurosur. Case Lessons2022416CASE2221410.3171/CASE2221436254353
    [Google Scholar]
  7. EwingJ. Classics in oncology. Diffuse endothelioma of bone. James Ewing. Proceedings of the New York Pathological Society, 1921.CA Cancer J. Clin.1972222959810.3322/canjclin.22.2.954622125
    [Google Scholar]
  8. YipC.M. HsuS.S. ChangN.J. WangJ.S. LiaoW.C. ChenJ.Y. LiuS.H. ChenC.H. Primary vaginal extraosseous Ewing sarcoma/primitive neuroectodermal tumor with cranial metastasis.J. Chin. Med. Assoc.200972633233510.1016/S1726‑4901(09)70381‑819541570
    [Google Scholar]
  9. PotratzJ. DirksenU. JürgensH. CraftA. Ewing sarcoma: Clinical state-of-the-art.Pediatr. Hematol. Oncol.201229111110.3109/08880018.2011.62203422295994
    [Google Scholar]
  10. WomerR.B. WestD.C. KrailoM.D. DickmanP.S. PawelB.R. GrierH.E. MarcusK. SailerS. HealeyJ.H. DormansJ.P. WeissA.R. Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: A report from the Children’s Oncology Group.J. Clin. Oncol.201230334148415410.1200/JCO.2011.41.570323091096
    [Google Scholar]
  11. BecerraB.J.E. RamírezN.L.F. ZúñigaL.A.J. GilT.N. ApazaR.R. Primary intracranial peripheral primitive neuroectodermal tumor: Lessons from an exceptionally rare neoplasm. Illustrative case.J. Neurosur. Case Lessons2024716CASE2413310.3171/CASE2413338621307
    [Google Scholar]
  12. DamerA. HamiltonL. TuA. JohnstonD.L. RamphalR. WilsonN. Primary intramedullary extradural Ewing sarcoma.Radiol. Case Rep.20241962381238910.1016/j.radcr.2024.02.10138572274
    [Google Scholar]
  13. AsriC.E.A. BenzagmoutM. ChakourK. ChaouiM.F. LaaguiliJ. ChahdiH. GazzazM. MostarchidE.B. Primary intracranial pPNET/Ewing sarcoma: Diagnosis, management, and prognostic factors dilemma–a systematic review of the literature.World Neurosurg.201811534635610.1016/j.wneu.2018.04.16429729469
    [Google Scholar]
  14. ChenJ. JiangQ. ZhangY. YuY. ZhengY. ChenJ. ZhaoY. MiaoZ. FanF. WangY. Clinical features and long-term outcome of primary intracranial Ewing sarcoma/peripheral primitive neuroectodermal tumors: 14 cases from a single institution.World Neurosurg.2019122e1606e161410.1016/j.wneu.2018.11.15130496933
    [Google Scholar]
  15. KambeA. KuwamotoS. ShimizuT. AmisakiH. SakamotoM. InagakiH. KurosakiM. A case of intracranial myxoid mesenchymal tumor with EWSR1:CREM fusion in an adult female: Extensive immunohistochemical evaluation.Neuropathology202141431532310.1111/neup.1274034258808
    [Google Scholar]
  16. ParidaD.K. MishraS.S. SenapatiS. PatraS. Multifocal Ewing′s sarcoma, presenting radiologically as extradural hematoma.Neurol. India201260336136210.4103/0028‑3886.9854622824715
    [Google Scholar]
  17. ZhangY. LiH. LiZ. LiuM. YangL. FanL. HuangC. LiB.S. Synthetic treatment of intracranial peripheral primitive neuroectodermal tumor with multiple metastasis: A case report.OncoTargets Ther.201693327333310.2147/OTT.S10398827330312
    [Google Scholar]
  18. RajeshA. VelivelaK. UppinM. PurohitA. Primary intracranial peripheral PNET" - A case report and review.Neurol. India201462666967310.4103/0028‑3886.14940025591683
    [Google Scholar]
  19. KumarV. SinghA. SharmaV. KumarM. Primary intracranial dural-based Ewing sarcoma/peripheral primitive neuroectodermal tumor mimicking a meningioma: A rare tumor with review of literature.Asian J. Neurosurg.201712335135710.4103/1793‑5482.18506028761507
    [Google Scholar]
  20. BiermannJ.S. Updates in the treatment of bone cancer.J. Natl. Compr. Canc. Netw.2013115S68168310.6004/jnccn.2013.020023704242
    [Google Scholar]
  21. AbboudA. MasrouhaK. SalibaM. HaidarR. SaabR. KhouryN. TawilA. SaghiehS. Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis (Review).Oncol. Lett.202121535410.3892/ol.2021.1261533747211
    [Google Scholar]
  22. HuangJ. GhentF. LevingstonR. ScholsemM. Intracranial Ewing Sarcoma – A case report.Surg. Neurol. Int.20201113410.25259/SNI_178_202032547821
    [Google Scholar]
  23. CasaliP.G. BielackS. AbecassisN. AroH.T. BauerS. BiaginiR. BonvalotS. BoukovinasI. BoveeJ.V.M.G. BrennanB. BrodowiczT. BrotoJ.M. BrugièresL. BuonadonnaA. ÁlavaD.E. TosD.A.P. MuroD.X.G. DileoP. DhoogeC. ErikssonM. FagioliF. FedenkoA. FerraresiV. FerrariA. FerrariS. FrezzaA.M. GasparN. GasperoniS. GelderblomH. GilT. GrignaniG. GronchiA. HaasR.L. HassanB. NoltingH.S. HohenbergerP. IsselsR. JoensuuH. JonesR.L. JudsonI. JutteP. KaalS. KagerL. KasperB. KopeckovaK. KrákorováD.A. LadensteinR. Le CesneA. LugowskaI. MerimskyO. MontemurroM. MorlandB. PantaleoM.A. PianaR. PicciP. NeumannP.S. PousaA.L. ReichardtP. RobinsonM.H. RutkowskiP. SafwatA.A. SchöffskiP. SleijferS. StacchiottiS. StraussS.J. HallS.K. UnkM. CoevordenV.F. van der GraafW.T.A. WhelanJ. WardelmannE. ZaikovaO. BlayJ.Y. Bone sarcomas: ESMO–PaedCan–EURACAN clinical practice guidelines for diagnosis, treatment and follow-up.Ann. Oncol.201829iv79iv9510.1093/annonc/mdy31030285218
    [Google Scholar]
  24. YangM.J. WhelanR. MaddenJ. LevyM.J.M. DeMastersK.B.K. HankinsonT.C. ForemanN.K. HandlerM.H. Intracranial Ewing sarcoma: Four pediatric examples.Childs Nerv. Syst.201834344144810.1007/s00381‑017‑3684‑729285586
    [Google Scholar]
  25. HaeuslerJ. RanftA. BoellingT. GoshegerG. MunzingerB.G. ViethV. BurdachS. van den BergH. JuergensH. DirksenU. The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES).Cancer2010116244345010.1002/cncr.2474019924786
    [Google Scholar]
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