Rhabdomyosarcoma classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Shadan Mehraban, M.D.[2]
Overview
Classification
Histologic classification of rhabdomyosarcoma
- The international classification of rhabdomyosarcoma (RMS) was created by the Intergroup Rhabdomyosarcoma Study group (IRSG) therapeutic trials (IRS-I to IV).[1][2]
- The four main subgroups are:
- Embryonal RMS
- Botryoid and spindle cell (leiomyomatous) RMS
- Sclerosing and spindle cell RMS with MyoD1 mutation
- Alveolar RMS
- Alveolar rhabdomyosarcoma
- Usually affects young adults
- Early metastasis is common
- Embryonal rhabdomyosarcoma
- Usually affects children <10 years of age
- Typically locally invasive
- Botryoid rhabdomyosarcoma
- Spindle cell rhabdomyosarcoma
- Anaplastic rhabdomyosarcoma
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.
References
- ↑ Qualman SJ, Coffin CM, Newton WA, Hojo H, Triche TJ, Parham DM; et al. (1998). "Intergroup Rhabdomyosarcoma Study: update for pathologists". Pediatr Dev Pathol. 1 (6): 550–61. PMID 9724344.
- ↑ Hicks J, Flaitz C (2002). "Rhabdomyosarcoma of the head and neck in children". Oral Oncol. 38 (5): 450–9. PMID 12110339.