CC BY-NC-ND 4.0 · Revista Chilena de Ortopedia y Traumatología 2023; 64(02): e109-e113
DOI: 10.1055/s-0043-1775833
Reporte de caso | Case Report

Case Report: Periosteal Ewing's sarcoma of the proximal humerus

Article in several languages: español | English
Hari Isvara Castro Herrán
1   Departamento de Ortopedia y Traumatología, Pontificia Universidad Católica de Chile, Santiago, Chile
María Emilia Riquelme Stagnaro
2   Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile
Tomás Zamora Helo
1   Departamento de Ortopedia y Traumatología, Pontificia Universidad Católica de Chile, Santiago, Chile
Laura Fonseca Muñoz
3   Departamento de Pediatría - Unidad Cuidados Intensivos, Pontificia Universidad Católica de Chile, Santiago, Chile
Eduardo Botello Correa
1   Departamento de Ortopedia y Traumatología, Pontificia Universidad Católica de Chile, Santiago, Chile
› Author Affiliations


Ewing's sarcoma is the third most common malignant bone tumor, occurring in patients under 20 years of age in 80% of cases. Histologically it is composed of small round cells with round nuclei with chromatin and eosinophilic cytoplasm. For its definitive diagnosis, a molecular study is necessary. It classically affects the medullary cavity of the diaphysis of long bones, however, it has less frequent presentations such as the extra osseous and the periosteal. We present a case report of a 17-year-old patient with swelling in the left proximal humerus with imaging studies compatible with a periosteal tumor lesion that spares the medullary cavity. A biopsy of the tumor was performed with histopathological and molecular confirmation of Ewing's sarcoma, which was treated with concomitant chemotherapy and radiotherapy, obtaining a progressive decrease in the size of the lesion and therefore surgery was not necessary. Periosteal Ewing's sarcoma is an extremely unusual entity, with few cases reported in the literature, which makes it important to recognize and treat these aggressive bone lesions in a timely manner.

Publication History

Received: 11 November 2022

Accepted: 14 August 2023

Article published online:
30 October 2023

© 2023. Sociedad Chilena de Ortopedia y Traumatologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (

Thieme Revinter Publicações Ltda.
Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil

  • Referencias

  • 1 Bedard J, Burns J, de Comas A. Periosteal Ewing Sarcoma in a 65-Year-Old Man: A Case Report. JBJS Case Connect 2017; 7 (02) e27
  • 2 Board WC of TE. Soft tissue and bone tumours [Internet]. 5th ed. series W classification of tumours, editor. Vol. 3. Lyon (France): International Agency for Research on Cancer; 2020 . Available from:
  • 3 Kollender Y, Shabat S, Nirkin A. et al. Periosteal Ewing's Sarcoma: Report of Two New Cases and Review of the Literature. Sarcoma 1999; 3 (02) 85-88
  • 4 Hatori M, Okada K, Nishida J, Kokubun S. Periosteal Ewing's sarcoma: radiological imaging and histological features. Arch Orthop Trauma Surg 2001; 121 (10) 594-597
  • 5 Savvidou O, Papakonstantinou O, Lakiotaki E. et al. Surface bone sarcomas: an update on current clinicopathological diagnosis and treatment. EFORT Open Rev 2021; 6 (10) 905-917
  • 6 Anderson WJ, Doyle LA. Updates from the 2020 World Health Organization Classification of Soft Tissue and Bone Tumours. Histopathology 2021; 78 (05) 644-657
  • 7 Choi JH, Ro JY. The 2020 WHO Classification of Tumors of Bone: An Updated Review. Adv Anat Pathol 2021; 28 (03) 119-138
  • 8 Sbaraglia M, Bellan E, Dei Tos AP. The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives. Pathologica 2021; 113 (02) 70-84
  • 9 Sherman RS, Soong KY. Ewing's sarcoma: its Roentgen classification and diagnosis. Radiology 1956; 66 (04) 529-539
  • 10 Violon F, Burns R, Mihoubi F. et al. Intramedullary, periosteal, and extraskeletal Ewing sarcomas: retrospective study of a series of 126 cases in a reference center. Skeletal Radiol 2022; 51 (08) 1659-1670
  • 11 Harper K, Sathiadoss P, Saifuddin A, Sheikh A. A review of imaging of surface sarcomas of bone. Skeletal Radiol 2021; 50 (01) 9-28
  • 12 Ahlawat S, Fayad LM. Revisiting the WHO classification system of bone tumours: emphasis on advanced magnetic resonance imaging sequences. Part 2. Pol J Radiol 2020; 85 (01) e409-e419
  • 13 Shapeero LG, Vanel D, Sundaram M. et al. Periosteal Ewing sarcoma. Radiology 1994; 191 (03) 825-831
  • 14 Hakozaki M, Hojo H, Tajino T. et al. Periosteal Ewing sarcoma family of tumors of the femur confirmed by molecular detection of EWS-FLI1 fusion gene transcripts: a case report and review of the literature. J Pediatr Hematol Oncol 2007; 29 (08) 561-565
  • 15 Bator SM, Bauer TW, Marks KE, Norris DG. Periosteal Ewing's sarcoma. Cancer 1986; 58 (08) 1781-1784
  • 16 Bernstein M, Kovar H, Paulussen M. et al. Ewing's sarcoma family of tumors: current management. Oncologist 2006; 11 (05) 503-519