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Extraskeletal Ewing sarcoma

Disease definition

Extraskeletal Ewing sarcoma is a rare, poorly differentiated, highly malignant, soft tissue tumor, derived from neuroectoderm, that is morphologically indistinguishable from skeletal Ewing sarcoma but is located in extraosseous locations, with the most common being: chest wall, paravertebral region, abdominopelvic area (with predilection for the retroperitoneal space), gluteal region and lower extremities. Clinical presentation is highly variable and depends on tumor localization. Local recurrence is common and metastatic disease most frequently involves the bones and lungs.

ORPHA:370334

Classification level: Disorder
  • Synonym(s):
    • EOE
    • Extraosseous Ewing sarcoma
    • Extraosseous Ewing tumor
    • Extraskeletal Ewing tumor
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Childhood, Adolescent, Adult
  • ICD-10: C49.9
  • OMIM: -
  • UMLS: C0279980  C0852530
  • MeSH: -
  • GARD: -
  • MedDRA: -

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The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.