Orphanet: Rabdomiosarcoma pleomorfo

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Pleomorphic rhabdomyosarcoma

Disease definition

A rare soft tissue sarcoma characterized by a high-grade lesion occurring almost exclusively in adults, composed of bizarre polygonal, round, and spindle cells with evidence of smooth muscle differentiation. Patients usually present with a rapidly growing, painful mass located in the deep soft tissues of the extremities, but also other anatomic regions. Prognosis is generally poor.


Classification level: Subtype of disorder
  • Synonym(s): -
  • Prevalence: <1 / 1 000 000
  • Inheritance: -
  • Age of onset: Infancy, Childhood, Adult, Adolescent, Elderly
  • ICD-10: C49.9
  • OMIM: -
  • UMLS: C0334480
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


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