Rhabdomyosarcoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Classification

Multiple classification systems have been proposed for subclassifying rhabdomyosarcomas. The most recent classification system, the "International Classification of Rhabdomyosarcoma", was created by the Intergroup Rhabdomyosarcoma Study.[1] This system attempts to combine elements of the previous systems and correlate these with prognosis based on tumor type. Rhabdomyosarcomas classified into six subtypes:

  • Botryoid rhabdomyosarcoma
  • Spindle cell rhabdomyosarcoma
  • Embryonal rhabdomyosarcoma
  • Alveolar rhabdomyosarcoma
  • Undifferentiated sarcoma

Several additional subtypes of rhabdomyosarcoma exist that do not fit into the International Classification scheme:

  • Pleomorphic rhabdomyosarcoma: Usually occurs in adults rather than children.
  • Sclerosing rhabdomyosarcoma: It is a rare rhabdomyosarcoma subtype recently characterized by Folpe, et al.; it is not included in this system.

References

  1. Newton WA, Gehan EA, Webber BL; et al. (September 1995). "Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification—an Intergroup Rhabdomyosarcoma Study". Cancer. 76 (6): 1073–85. doi:10.1002/1097-0142(19950915)76:6<1073::AID-CNCR2820760624>3.0.CO;2-L. PMID 8625211.

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