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Intramedullary, periosteal, and extraskeletal Ewing sarcomas: retrospective study of a series of 126 cases in a reference center

Skeletal Radiology volume 51, pages 1659–1670 (2022)Cite this article

Abstract

Objective

To evaluate the proportion of extraskeletal, periosteal, and intramedullary Ewing sarcomas among musculoskeletal Ewing sarcomas.

Material and method

Our single-center retrospective study included patients with musculoskeletal Ewing sarcoma diagnosed between 2005 and 2019 in our pathology center (cases from our adult bone tumor referral center and adult and pediatric cases referred for review). Recurrences, metastases, and visceral Ewing sarcomas were excluded. Intramedullary Ewing sarcomas were defined by involvement of the medullary cavity. Periosteal cases were defined by involvement of the subperiosteal area without extension to the medullary cavity. Extraskeletal cases were defined by the absence of involvement of the bone tissue and the subperiosteal area.

Results

Our series included 126 patients with musculoskeletal Ewing sarcoma, including 118 skeletal Ewing sarcomas (93.7%) and 8 extraskeletal Ewing sarcomas (6.3%). Of the 118 skeletal Ewing sarcomas 112 were intramedullary (88.9%) and 6 were periosteal (4.8%). Extraskeletal Ewing sarcomas were more common in women and in patients older than 40 (p < 0.05).

Discussion

The 6.3% proportion of extraskeletal Ewing sarcoma is lower than the median of 30% estimated from the literature. This difference could be explained by an overestimation of extraskeletal Ewing sarcomas of the chest wall (Askin tumors), an underestimation of periosteal cases confused with extraskeletal cases, and the presence of “Ewing-like” soft tissue sarcomas in previous series. Because of its prognostic and therapeutic impact, the distinction of morphologic subtypes requires the cooperation of experienced radiologists and pathologists.

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Author notes

  1. Violon F and Burns R contributed equally to the manuscript

Authors and Affiliations

  1. Service de Pathologie, Hôpital Cochin, AP‑HP, Paris, France

    F. Violon, V. Audard & F. Larousserie

  2. Faculté de Médecine de Nancy, Université de Lorraine, Nancy, France

    F. Violon

  3. Service de Radiologie, Hôpital Cochin, AP-HP, Paris, France

    R. Burns, F. Mihoubi & A. Feydy

  4. Université de Paris, Paris, France

    R. Burns, D. Biau, A. Feydy & F. Larousserie

  5. Service de Chirurgie Orthopédique, Hôpital Cochin, AP-HP, Paris, France

    D. Biau

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Approval from the local ethic comity (registration number #AAA-2020–08040) and the local data protection office (registration number #20210215115018) was obtained and in keeping with the policies for a retrospective review, informed consent was not required.

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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 51, 1659–1670 (2022). https://doi.org/10.1007/s00256-021-03983-6

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