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Evolving classification of rhabdomyosarcoma

Journal

HISTOPATHOLOGY
Volume 80, Issue 1, Pages 98-108

Publisher

WILEY
DOI: 10.1111/his.14449

Keywords

alveolar; embryonal; FOXO1; MYOD1; pleomorphic; rhabdomyosarcoma; spindle cell; sclerosing

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This article discusses the evolution of the classification of rhabdomyosarcoma, the histomorphological and genetic features of each subtype, and the diagnostic approach to these tumors.
Rhabdomyosarcomas comprise the single largest category of soft tissue sarcomas in children and adolescents in the United States, occurring in 4.5 million people aged below 20 years. Based on the clinicopathological features and genetic abnormalities identified, rhabdomyosarcomas are classified into embryonal, alveolar, spindle cell/sclerosing and pleomorphic subtypes. Each subtype shows distinctive morphology and has characteristic genetic abnormalities. This review discusses the evolution of the classification of rhabdomyosarcoma to the present day, together with a discussion of key histomorphological and genetic features of each subtype and the diagnostic approach to these tumours.

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