Acute clinical manifestation of Ewing sarcoma spreading from the second cervical vertebra

Objective. Ewing sarcoma is a highly metastatic form of sarcoma, the second most common primary malignant bone tumor, primarily affecting adolescents. Its extreme rarity in the cervical spine may lead to a delay in diagnosis until the tumor reaches an advanced stage and compresses the spinal cord. Initial symptoms may include neck pain and radiculopathy. These symptoms may progress rapidly, accompanied by limb weakness and bladder or bowel dysfunction, developing within days over the disease onset. A high degree of clinical suspicion is crucial for early diagnosis and treatment. The aim: To present a clinical description of a case of acute clinical manifestation of Ewing’s sarcoma extending from the second cervical vertebra.
Materials and methods. We describe a case of a 3-year-old girl without history of chronic illness or trauma. Clinical manifestations of symptoms began 6 months before the full-blown clinical picture, with mild, progressive, intermittent pain in the cervical spine, initially attributed to torticollis. Three months later, with persistent symptoms, the patient underwent a cervical spine X-ray, which at that time revealed no significant pathological changes. Approximately 6 months after onset, symptoms sharply worsened over several days, leading to severe pain, tetraparesis, and pelvic dysfunction. MRI revealed a lesion of the second cervical vertebra, affecting both the vertebral body and the posterior arch, with infiltration of the anterior epidural space, nerve root openings at the level of the second and third cervical vertebrae, and adjacent paravertebral muscles. Tumor invasion of the right vertebral artery encompassed it from all sides. A chest CT scan revealed multiple metastatic lesions in the lungs (approximately 25 metastases in total).
Results. The patient underwent emergency surgery, including a laminectomy from the 2nd to 4th cervical vertebrae with partial resection of the extradural soft tissue component of the tumor. Postoperatively, almost complete regression of neurological deficits was noted, with a score of 4.5 in the legs and 4.5 in the arms, as well as regression of pelvic abnormalities. The patient was transferred to the Pediatric Oncology Department of the Dmitry Rogachev National Medical Research Center for Pediatric Hematology, Oncology, and Immunology, where multimodal chemotherapy and radiation therapy were initiated.
Conclusion. Primary Ewing sarcomas affecting the C2 vertebra are extremely rare. This location presents diagnostic challenges due to its atypical clinical presentation and radiographic and histopathological similarities to other tumors. This case highlights the importance of a multidisciplinary approach for accurate diagnosis and optimal treatment. More accurate recognition of these rare forms can facilitate early diagnosis and improve patient prognosis.
Contribution: Rynda A.Yu., Vorozhtsov I.N., Grachev N.S., Sanakoeva A.V., Tarakanova A.V. — study concept and design; Rynda A.Yu., Papusha L.I., Vorozhtsov I.N., Sanakoeva A.V., Tarakanova A.V., Tereshchenko G.V. — data collection and processing, statistical processing; Rynda A.Yu., Karachunsky A.I., Sanakoeva A.V., Tarakanova A.V., Tereshchenko G.V. — writing the text; Rynda A.Yu., Papusha L.I., Grachev N.S., Lopatin A.V., Karachunsky A.I. — scientific editing. All co-authors approved the final version of the article and are responsible for the integrity of all parts of the article.
Acknowledgments. The study had no sponsorship.
Conflict of interest. The authors declare no conflict of interest.

Received: January 19, 2026
Accepted: February 10, 2026
Published: February 27, 2026

1. Zöllner S.K., Amatruda J.F., Bauer S., Collaud S., de Álava E., DuBois S.G., et al. Ewing sarcoma-diagnosis, treatment, clinical challenges and future perspectives. J. Clin. Med. 2021; 10(8): 1685. https://doi.org/10.3390/jcm10081685

2. Strauss S.J., Berlanga P., McCabe M.G. Emerging therapies in Ewing sarcoma. Curr. Opin. Oncol. 2024; 36(4): 297–304. https://doi.org/10.1097/CCO.0000000000001048

3. Gulati D., Aggarwal A.N., Kumar S., Chaturvedi S. Primary Ewing’s sarcoma of the second cervical vertebra: A rare entity. J. Pediatr. Orthop. B. 2011; 20(6): 408–12. https://doi.org/10.1097/bpb.0b013e328345d78a

4. Giakoumettis D., Paraskeva K., Nikas I., Georgoulis G., Sfakianos G., Themistocleous M.S. Primary Ewing sarcoma of the axis-C2: A case report and the review of the literature. Neurol. Neurochir. Pol. 2018; 52(4): 534–42. https://doi.org/10.1016/j.pjnns.2018.02.005

5. Elaitari K., Jabour S., Boujida N., Allali N., Chat L., Kababri M.E.L., et al. Primary Ewing’s sarcoma of the cervical vertebra: A case report. Radiol. Case Reports. 2025; 20(10): 5116–21. https://doi.org/10.1016/j.radcr.2025.06.055

6. Shaker K., Alomar K., Wahoob M., Altabaa K., Omran M., Marwa G. A rare case of Ewing’s sarcoma primary of the cervical spine in a 1-year-old child: A case report and literature review. Int. J. Surg. Case Rep. 2024; 122: 110020. https://doi.org/10.1016/j.ijscr.2024.110020

7. Cherraqi A., Lemrabet A., Dokal I.D., Lrhorfi N., Belghiti H., Allali N., et al. Primary Ewing’s sarcoma of the spine: about a case. Glob. Pediatr. Health. 2022; 9: 2333794X221123874. https://doi.org/10.1177/2333794X221123874

8. Ansari M.N., Gupta A., Jain D.K., Rana D. The Ewing’s sarcoma of cervical spine-a rare occurrence. Spinal Cord Ser. Cases. 2022; 8(1): 68. https://doi.org/10.1038/s41394-022-00534-6

9. Sohrabi C., Mathew G., Maria N., Kerwan A., Franchi T., Agha R.A. The SCARE 2023 guideline: updating consensus Surgical CAse REport (SCARE) guidelines. Int. J. Surg. 2023; 109(5): 1136–40. https://doi.org/10.1097/JS9.0000000000000373

10. Mousavi S., Keykhosravi E., Rezaee H., Dehghanian P., Ebrahimzadeh F., Tavallayiet A. Giant primary epidural extraskeletal Ewing sarcoma in cervical spine of an infant: case report and review of the literature. Iran J. Child. Neurol. 2024; 18(4): 127–34. https://doi.org/10.22037/ijcn.v18i4.29422

11. Chechev E.I., Grachev N.S., Lopatin A.V., Vorozhtsov I.N., Babaskina N.V., Markov N.M. Osteosarcoma and Ewing’s sarcoma of the maxillofacial bones in children. Stomatologiya: teoriya i praktika. 2024; 2(1): 5–8. https://elibrary.ru/wowtpf (in Russian)

12. Khatavi A., Dhillon C.S., Chhasatia N. Primary Ewing’s sarcoma of the C2 vertebra with progressive quadriparesis: Report of a rare case and review of the literature. Surg. Neurol. Int. 2020; 11: 340. https://doi.org/10.25259/SNI_497_2020

13. Rynda A.Y., Olyushin V.E., Rostovtsev D.M., Kukanov K.K., Sklyar S.S., Zabrodskaya Yu.M. Patients with long-term survival in malignant gliomas after photodynamic therapy. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova. 2024; 124(6): 54–61. https://doi.org/10.17116/jnevro202412406154 https://elibrary.ru/nqihhs (in Russian)

14. Patel D.S., Desai R.T., Pandya A.J., Kothari N.R., Patel R., Chorawala M.R. Harnessing the immune responses: a new frontier in Ewing sarcoma treatment. Med. Oncol. 2025; 42(8): 291. https://doi.org/10.1007/s12032-025-02848-5

15. Rynda A.Y., Rostovthev D.M., Zabrodskaya Y.M., Olyushin V.E. Immunotherapy with autologous dendritic cells in the complex treatment of malignant gliomas – results. J. Neurooncol. 2024; 166(2): 309–19. https://doi.org/10.1007/s11060-023-04559-1

16. Mata Fernández C., Sebio A., Orcajo Rincón J., Martín Broto J., Martín Benlloch A., Marcilla Plaza D., et al. Clinical practice guidelines for the treatment of Ewing sarcoma (Spanish Sarcoma Research Group-GEIS). Clin. Transl. Oncol. 2025; 27(3): 824–36. https://doi.org/10.1007/s12094-024-03602-5

17. Bhattacharjee S., Venkata S.R., Uppin M.S. Skull and Spinal Ewing’s sarcoma in children: an institutional study. J. Pediatr. Neurosci. 2018; 13(4): 392–97. https://doi.org/10.4103/JPN.JPN_109_18

18. Clemente E.J.I., Navarro O.M., Navallas M., Ladera E., Torner F., Sunol M., et al. Multiparametric MRI evaluation of bone sarcomas in children. Insights Imaging. 2022; 13(1): 33. https://doi.org/10.1186/s13244-022-01177-9

19. Rynda A.Yu., Rostovtsev D.M., Olyushin V.E., Zabrodskaya Yu.M. Therapeutic pathomorphosis in malignant glioma tissues after photodynamic therapy with chlorin e6 (reports of two clinical cases). Biomed. Photon. 2020; 9(2): 45–54. https://doi.org/10.24931/2413-9432-2020-9-2-45-54

20. Brennan B., Kirton L., Marec-Bérard P., Gaspar N., Laurence V., Martín-Brotoet J., et al. Comparison of two chemotherapy regimens in patients with newly diagnosed Ewing sarcoma (EE2012): an open-label, randomised, phase 3 trial. Lancet. 2022; 400(10362): 1513–21. https://doi.org/10.1016/S0140-6736(22)01790-1

21. Duffaud F., Blay J.Y., Le Cesne A., Chevreau C., BoudouRouquette P., Kalbacher E., et al. Regorafenib in patients with advanced Ewing sarcoma: results of a non-comparative, randomised, double-blind, placebo-controlled, multicentre Phase II study. Br. J. Cancer. 2023; 129(12): 1940–48. https://doi.org/10.1038/s41416-023-02413-9

22. Italiano A., Mir O., Mathoulin-Pelissier S., Penel N., PipernoNeumann S., Bompas E., et al. Cabozantinib in patients with advanced Ewing sarcoma or osteosarcoma (CABONE): a multicentre, single-arm, phase 2 trial. Lancet Oncol. 2020; 21(3): 446–55. https://doi.org/10.1016/S1470-2045(19)30825-3

23. Tikhonova M.V., Budanov O.I., Slinin A.S., Bolshakov N.A., Melcher I., Remizov A.V., et al. Ewing’s sarcoma in children: a multidisciplinary approach to diagnostics and treatment. Pediatriya. 2018; 97(4): 37–50. https://doi.org/10.24110/0031-403X-2018-97-4-37-50 https://elibrary.ru/xtuurv (in Russian)

24. Sanakoeva A.V., Samarin A.E., Konopleva E.I., Tarakanova A.V., Sidorov I.V., Druy A.E., et al. Surgical treatment of intracerebral metastases of Ewing sarcoma in children. Case report and review. Voprosy neirokhirurgii im. N.N. Burdenko. 2025; 89(2): 92–6. https://doi.org/10.17116/neiro20258902192 https://elibrary.ru/oyqipa (in Russian)

25. Zhao D.Y., Zhang W.L., Cui Y., Ma C.B., Ni S.H., Zhang Z.Y. Primary Ewing’s sarcoma of the vertebral body: A case report. Medicine. 2019; 98(24): 15905. https://doi.org/10.1097/MD.0000000000015905

26. Kachanov D.Y., Abdullaev R.T., Kruchko N.V., Shamanskaya T.V., Inyushkina E.V., Dobrenkov K.V., et al. Second malignant neoplasms in children and adolescents in Moscow region. Onkogematologiya. 2009; 4(3): 34–40. https://elibrary.ru/mnibyv (in Russian)