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J Neurol Surg A Cent Eur Neurosurg 2024; 85(02): 215-220
DOI: 10.1055/s-0043-1776264
Case Report

Long-Term Survival after Primary Ewing's Sarcoma of the Skull with Intracranial Extension

Nikolai G. Rainov
1   MVZ Wirbelsäulenzentrum München-Taufkirchen, München, Germany
2   Department of Neurosurgery, University Hospital Heart and Brain, Pleven, Bulgaria
,
Dimitar Haritonov
2   Department of Neurosurgery, University Hospital Heart and Brain, Pleven, Bulgaria
,
Volkmar Heidecke
3   Department of Neurosurgery, Universitätsklinikum Augsburg, Augsburg, Germany
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Abstract

Background and Objective Primary Ewing's sarcoma of the skull is a very rare malignant neoplasm, predominantly occurring in children and adolescents. We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the standards of therapy for this type of tumor.

Clinical Presentation This 18-year-old male patient presented with a primary Ewing's sarcoma of the skull, involving the dura of the frontal and parietal lobes of the left cerebral hemisphere. He was treated with gross total surgical excision of tumor, skull reconstruction, chemotherapy, and irradiation. Twelve years after the surgery, the patient has no evidence of local recurrence or distant metastases. Radical surgical excision of the primary tumor with safety margins is thought to play a role in the favorable clinical course.

Conclusion The presented case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull bone. This rare type of tumor may allow better survival rates under adequate management than sarcoma elsewhere in the body.

Keywords

Ewing's sarcoma - cranial sarcoma - malignant bone tumor


Publication History

Received: 22 September 2019

Accepted: 13 February 2020

Article published online:
08 November 2023

© 2023. Thieme. All rights reserved.

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  • References

  • 1 Martin E, Senders JT, Ter Wengel PV, Smith TR, Broekman MLD. Treatment and survival of osteosarcoma and Ewing sarcoma of the skull: a SEER database analysis. Acta Neurochir (Wien) 2019; 161 (02) 317-325
    CrossrefPubMedGoogle Scholar
  • 2 Martin E, Radomski S, Harley EH. Pediatric Ewing sarcoma of the head and neck: A retrospective survival analysis. Int J Pediatr Otorhinolaryngol 2019; 117: 138-142
    CrossrefPubMedGoogle Scholar
  • 3 Bhattacharjee S, Venkata SR, Uppin MS. Skull and spinal Ewing's sarcoma in children: an institutional study. J Pediatr Neurosci 2018; 13 (04) 392-397
    CrossrefPubMedGoogle Scholar
  • 4 Bouaoud J, Temam S, Cozic N. et al. Ewing's Sarcoma of the head and neck: margins are not just for surgeons. Cancer Med 2018; 7 (12) 5879-5888
    CrossrefPubMedGoogle Scholar
  • 5 Hadfield MG, Quezado MM, Williams RL, Luo VY. Ewing's family of tumors involving structures related to the central nervous system: a review. Pediatr Dev Pathol 2000; 3 (03) 203-210
    CrossrefPubMedGoogle Scholar
  • 6 Yang MJ, Whelan R, Madden J. et al. Intracranial Ewing sarcoma: four pediatric examples. Childs Nerv Syst 2018; 34 (03) 441-448
    CrossrefPubMedGoogle Scholar
  • 7 Moschovi M, Alexiou GA, Tourkantoni N. et al. Cranial Ewing's sarcoma in children. Neurol Sci 2011; 32 (04) 691-694
    CrossrefPubMedGoogle Scholar
  • 8 Desai KI, Nadkarni TD, Goel A, Muzumdar DP, Naresh KN, Nair CN. Primary Ewing's sarcoma of the cranium. Neurosurgery 2000; 46 (01) 62-68 , discussion 68–69
    CrossrefPubMedGoogle Scholar
  • 9 Choi SW, Ko H. Primary Ewing sarcoma of the squamous temporal bone with intracranial and extracranial extension: a rare cause of sudden sensorineural hearing loss. Head Neck 2019; 41 (03) E38-E41
    PubMedGoogle Scholar
  • 10 Woo CG, Lee B, Song JS, Cho KJ. Clinicopathologic features of the non-CNS primary Ewing sarcoma family of tumors in the head and neck region. Appl Immunohistochem Mol Morphol 2018; 26 (09) 632-639
    CrossrefPubMedGoogle Scholar
  • 11 Salunke PS, Gupta K, Malik V. et al. Primary Ewing's sarcoma of cranial bones: analysis of ten patients. Acta Neurochir (Wien) 2011; 153 (07) 1477-1485
    CrossrefPubMedGoogle Scholar
  • 12 Erol FS, Ozveren MF, Ozercan IH, Topsakal C, Akdemir I. Primary Ewing's sarcoma of the occipital bone: case report. Neurol Med Chir (Tokyo) 2001; 41 (04) 206-209
    CrossrefPubMedGoogle Scholar
  • 13 Bhatoe HS, Deshpande GU. Primary cranial Ewing's sarcoma. Br J Neurosurg 1998; 12 (02) 165-169
    CrossrefPubMedGoogle Scholar
  • 14 Watanabe H, Tsubokawa T, Katayama Y, Koyama S, Nakamura S. Primary Ewing's sarcoma of the temporal bone. Surg Neurol 1992; 37 (01) 54-58
    CrossrefPubMedGoogle Scholar
  • 15 Krishnamani K, Kumar TN, Gandhi LV, Raghunadharao D, Sadashivudu G, Megha U. Primary Ewing's sarcoma of the cranium: case series and review of literature. J Cancer Res Ther 2014; 10 (02) 377-380
    CrossrefPubMedGoogle Scholar
  • 16 Berger M, Fagioli F, Abate M. et al. Unusual sites of Ewing sarcoma (ES): a retrospective multicenter 30-year experience of the Italian Association of Pediatric Hematology and Oncology (AIEOP) and Italian Sarcoma Group (ISG). Eur J Cancer 2013; 49 (17) 3658-3665
    CrossrefPubMedGoogle Scholar
  • 17 Kawano H, Nitta N, Ishida M, Fukami T, Nozaki K. Primary pericranial Ewing's sarcoma on the temporal bone: a case report. Surg Neurol Int 2016; 7 (Suppl. 15) S444-S448
    PubMedGoogle Scholar
  • 18 Castle M, Rivero M, Marquez J. Primary Ewing's sarcoma of the skull: radical resection and immediate cranioplasty after chemotherapy. A technical note. Childs Nerv Syst 2013; 29 (02) 303-306
    CrossrefPubMedGoogle Scholar
  • 19 Carlotti Jr CG, Drake JM, Hladky JP, Teshima I, Becker LE, Rutka JT. Primary Ewing's sarcoma of the skull in children. Utility of molecular diagnostics, surgery and adjuvant therapies. Pediatr Neurosurg 1999; 31 (06) 307-315
    CrossrefPubMedGoogle Scholar
  • 20 Grier HE, Krailo MD, Tarbell NJ. et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 2003; 348 (08) 694-701
    CrossrefPubMedGoogle Scholar