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Journals / Turkish Journal of Pediatrics / 2021 / Cilt: 63 - Sayı: 6

Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature

Eren MÜNGEN
Nilgün KURUCU
Tezer KUTLUK
Kader K. OĞUZ
Figen SÖYLEMEZOĞLU
Bilgehan YALÇIN
Pages
1084–1090
DOI
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

The prognosis is worse than other extraosseous Ewing sarcoma

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Primary spinal, intradural, extramedullary Ewing sarcoma (PSIEES) is exceptionally uncommon.
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Key Findings & Data: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.
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Conclusion & Impact:The prognosis is worse than other extraosseous Ewing sarcoma
🔬Design:Review & Systematic Survey
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Özet

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

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Cite This Article

MÜNGEN, E., KURUCU, N., KUTLUK, T., OĞUZ, K. K., SÖYLEMEZOĞLU, F., YALÇIN, B. (2021). Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature. Turkish Journal of Pediatrics, Cilt: 63 - Sayı: 6, 1084–1090. https://ajindex.com/articles/411511

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References (12)

  1. Riggi N, Stamenkovic I. The Biology of Ewing sarcoma. Cancer Lett 2007
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. https://doi.org/10.1080/08880010390220126 7. Koeller KK, Shih RY. Intradural extramedullary spinal neoplasms: radiologic-pathologic correlation. Radiographics 2019
  8. 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
  9. 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. 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
  11. 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
  12. 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