Background. Primary spinal, intradural, extramedullary Ewing sarcoma (PSIEES) is exceptionally uncommon. Here, we present an interesting pediatric case with a PSIEES diagnosis confirmed by the presence of a specific fusion protein in the tumor tissue and who then developed a cerebellar recurrence. We also reviewed the PSIEES cases in childhood reported in the literature. Case. An 8.5-year-old boy was admitted to a local hospital with a one-month history of severe back and limb pain, and inability to move his lower limbs. Physical examination revealed paraparesis in the lower extremities. Spinal MRI revealed multiple intradural extramedullary masses at the L2-L3, L4-5 and L5-S1 levels. He underwent surgery and near total excision of all three masses were performed. Histopathological diagnosis of Ewing Sarcoma was confirmed with EWS-ERG gene rearrangement. The patient was treated according to EuroEwing chemotherapy protocol. A total dose of 4500 cGy radiotherapy was applied to the tumor location at L2-S1 paravertebral region. Eighteen months after the end of treatment, a mass in the left cerebellar hemisphere was determined. Gross total excision was performed. Histopathological examination of the tumor showed Ewing sarcoma. Radiological screening revealed isolated central nervous system recurrence. A total of 4500 cGy radiotherapy was applied. He is on a second-line treatment consisting of gemcitabine and docetaxel without any evidence of disease. Conclusions. Ewing Sarcoma with spinal intradural region in childhood is very rare. We could only find 17 pediatric cases reported in the literature. Neurological findings occur earlier in tumors of this region. The prognosis is worse than other extraosseous Ewing sarcoma
___
1. Riggi N, Stamenkovic I. The Biology of Ewing sarcoma. Cancer Lett 2007; 254: 1-10. https://doi. org/10.1016/j.canlet.2006.12.009
2. Bernstein M, Kovar H, Paulussen M, et al. Ewing’s sarcoma family of tumors: current management. Oncologist 2006; 11: 503-519. https://doi.org/10.1634/ theoncologist.11-5-503
3. Javery O, Krajewski K, O’Regan K, et al. A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol 2011; 197: W1015-W1022. https:// doi.org/10.2214/AJR.11.6667
4. Yan Y, Xu T, Chen J, Hu G, Lu Y. Intraspinal Ewing’s sarcoma/primitive neuroectodermal tumors. J Clin Neurosci 2011; 18: 601-606. https://doi.org/10.1016/j. jocn.2010.09.012
5. Lu VM, Goyal A, Alvi MA, Kerezoudis P, Haddock MG, Bydon M. Primary intradural Ewing’s sarcoma of the spine: a systematic review of the literature. Clin Neurol Neurosurg 2019; 177: 12-19. https://doi. org/10.1016/j.clineuro.2018.12.011
6. Pollono D, Tomarchia S, Drut R, Ibanez O, Ferreyra M, Cedola J. Spinal cord compression: a review of 70 pediatric patients. Pediatr Hematol Oncol 2003; 20: 457-466. https://doi.org/10.1080/08880010390220126
7. Koeller KK, Shih RY. Intradural extramedullary spinal neoplasms: radiologic-pathologic correlation. Radiographics 2019; 39: 468-490. https://doi. org/10.1148/rg.2019180200
8. Hisaoka M, Hashimoto H, Murao T. Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina. Virchows Arch 1997; 431: 365-369. https:// doi.org/10.1007/s004280050112
9. Uesaka T, Amano T, Inamura T, et al. Intradural, extramedullary spinal Ewing’s sarcoma in childhood. J Clin Neurosci 2003; 10: 122-125. https:// doi.org/10.1016/S0967-5868(02)00279-5
10. Harimaya K, Oda Y, Matsuda S, Tanaka K, Chuman H, Iwamoto Y. Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. Spine (Phila Pa 1976) 2003; 28: E408-E412. https://doi.org/10.1097/01. BRS.0000085099.47800.DF
11. Woestenborghs H, Debiec-Rychter M, Renard M, et al. Cytokeratin-positive meningeal peripheral PNET/Ewing’s sarcoma of the cervical spinal cord: diagnostic value of genetic analysis. Int J Surg Pathol 2005; 13: 93-97. https://doi. org/10.1177/106689690501300114
12. Perry R, Gonzales I, Finlay J, Zacharoulis S. Primary peripheral primitive neuroectodermal tumors of the spinal cord: report of two cases and review of the literature. J Neurooncol 2007; 81: 259-264. https:// doi.org/10.1007/s11060-006-9178-1
13. Klimo P Jr, Codd PJ, Grier H, Goumnerova LC . Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr 2009; 4: 222-229. https:// doi.org/10.3171/2009.3.PEDS08272
14. Duan XH, Ban XH, Liu B, et al. Intraspinal primitive neuroectodermal tumor: imaging findings in six cases. Eur J Radiol. 2011; 80: 426-431. https://doi. org/10.1016/j.ejrad.2010.06.005
15. Wu G, Ghimire P, Zhu L, Baral A, Su J. Magnetic resonance imaging characteristics of primary intraspinal peripheral primitive neuroectodermal tumour. Can Assoc Radiol J 2013; 64: 240-245. https:// doi.org/10.1016/j.carj.2012.01.001
16. Zhao M, Zhang B, Liang F, Zhang J. Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: case report and review of the literature. Int J Clin Exp Pathol 2014; 7: 9081- 9085.
17. Kartal A, Akatli A. Primary intradural extraosseous Ewing’s sarcoma in a young child. Childs Nerv Syst 2016; 32: 409-410. https://doi.org/10.1007/s00381-016- 3020-7
18. Chen F, Chiou SS, Lin SF, Lieu AS, Chen YT, Huang CJ. Recurrent spinal primitive neuroectodermal tumor with brain and bone metastases: a case report. Medicine (Baltimore) 2017; 96: e8658. https://doi. org/10.1097/MD.0000000000008658
19. Scantland JT, Gondim MJ, Koivuniemi AS, Fulkerson DH, Shih CS. Primary spinal intradural extraosseous Ewing sarcoma in a pediatric patient: case report and review of the literature. Pediatr Neurosurg 2018; 53: 222-228. https://doi.org/10.1159/0004887
0. Yi X, Zhou M, Liao S, et al. Spinal peripheral primitive neuroectodermal tumors: a radiological analysis of ten cases. Turk Neurosurg 2019; 29: 33- 42. https://doi.org/10.5137/1019-5149.JTN.21931-17.2
21. Applebaum MA, Worch J, Matthay KK, et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer 2011; 117: 3027-3032. https:// doi.org/10.1002/cncr.25840
22. Cash T, McIlvaine E, Krailo MD, et al. Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: a report from the Children’s Oncology Group. Pediatr Blood Cancer 2016; 63: 1771-1779. https://doi.org/10.1002/pbc.26096
23. Paterakis KN, Brotis A, Dardiotis E, Giannis T, Tzerefos C, Fountas KN. Multimodality treatment of intradural extramedullary Ewing’s sarcomas. A systematic review. Clin Neurol Neurosurg 2018; 164: 169-181. https://doi.org/10.1016/j. clineuro.2017.11.014
24. Shuper A, Cohen IJ, Mor C, Ash S, Kornreich L, Zaizov R. Metastatic brain involvement in Ewing family of tumors in children. Neurology 1998; 51: 1336-1338. https://doi.org/10.1212/WNL.51.5.1336
25. Parasuraman S, Langston J, Rao BN, et al. Brain metastases in pediatric Ewing sarcoma and rhabdomyosarcoma: the St. Jude Children’s Research Hospital experience. J Pediatr Hematol Oncol 1999; 21: 370-377. https://doi.org/10.1097/00043426- 199909000-00007
26. Haresh KP, Chinikkatti SK, Prabhakar R, et al. A rare case of intradural extramedullary Ewing’s sarcoma with skip metastasis in the spine. Spinal Cord 2008; 46: 582-584. https://doi.org/10.1038/sc.2008.8
27. Gong HS, Huang QS, Liu GJ, Chen FH, Zhao HB. Cervical primary Ewing’s sarcoma in intradural and extramedullary location and skip metastasis to cauda equina. Turk Neurosurg 2015; 25: 943-947. https://doi.org/10.5137/1019-5149.JTN.12025-14.