Rhabdomyosarcoma classification: Difference between revisions
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{{CMG}};{{AE}} {{S.M}} | {{CMG}};{{AE}} {{S.M}} | ||
==Overview== | ==Overview== | ||
The international classification of rhabdomyosarcoma (RMS) was created by the Intergroup Rhabdomyosarcoma Study group (IRSG). The | The international classification of rhabdomyosarcoma (RMS) was created by the Intergroup [[Rhabdomyosarcoma]] Study group (IRSG). The four main subgroups include: [[Embryonal rhabdomyosarcoma]] (ERMS), [[Alveolar rhabdomyosarcoma]] (ARMS), [[Botryoid rhabdomyosarcoma|Botryoid]] and [[spindle cell]] (leiomyomatous) RMS, Sclerosing and [[spindle cell]] RMS. The other separate categories for subtypes which do not classify into above groups are [[Undifferentiated]], [[Pleomorphic]]/[[anaplastic]], and [[Sarcoma]], not otherwise specified (NOS). | ||
==Classification== | ==Classification== | ||
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| style="background:#DCDCDC;" align="center" + |[[Embryonal rhabdomyosarcoma]] (ERMS) | | style="background:#DCDCDC;" align="center" + |[[Embryonal rhabdomyosarcoma]] (ERMS) | ||
| style="background:#F5F5F5;" +| | | style="background:#F5F5F5;" + | | ||
* The most common group | * The most common group | ||
* Contains 59% of all RMS | * Contains 59% of all RMS | ||
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| style="background:#DCDCDC;" align="center" + |[[Alveolar rhabdomyosarcoma]] (ARMS) | | style="background:#DCDCDC;" align="center" + |[[Alveolar rhabdomyosarcoma]] (ARMS) | ||
| style="background:#F5F5F5;" +| | | style="background:#F5F5F5;" + | | ||
* Determined by [[FOXO1]] [[rearrangement]], t (1;13) or t (2;13)<ref name="pmid12951587">{{cite journal| author=Helman LJ, Meltzer P| title=Mechanisms of sarcoma development. | journal=Nat Rev Cancer | year= 2003 | volume= 3 | issue= 9 | pages= 685-94 | pmid=12951587 | doi=10.1038/nrc1168 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12951587 }}</ref> | * Determined by [[FOXO1]] [[rearrangement]], t (1;13) or t (2;13)<ref name="pmid12951587">{{cite journal| author=Helman LJ, Meltzer P| title=Mechanisms of sarcoma development. | journal=Nat Rev Cancer | year= 2003 | volume= 3 | issue= 9 | pages= 685-94 | pmid=12951587 | doi=10.1038/nrc1168 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12951587 }}</ref> | ||
* Poorer [[prognosis]] | * Poorer [[prognosis]] | ||
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| style="background:#DCDCDC;" align="center" + |[[Botryoid rhabdomyosarcoma|Botryoid]] and [[spindle cell]] RMS | | style="background:#DCDCDC;" align="center" + |[[Botryoid rhabdomyosarcoma|Botryoid]] and [[spindle cell]] RMS | ||
| style="background:#F5F5F5;" +| | | style="background:#F5F5F5;" + | | ||
* [[Rhabdomyosarcoma|Botryoid]] RMS contain 6% of all RMS | * [[Rhabdomyosarcoma|Botryoid]] RMS contain 6% of all RMS | ||
* [[Spindle cell]] RMS contains 3% of all RMS | * [[Spindle cell]] RMS contains 3% of all RMS | ||
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| style="background:#DCDCDC;" align="center" + |Sclerosing RMS | | style="background:#DCDCDC;" align="center" + |Sclerosing RMS | ||
| style="background:#F5F5F5;" +| | | style="background:#F5F5F5;" + | | ||
* Less common variants of ERMS | * Less common variants of ERMS | ||
* Poor outcome | * Poor outcome | ||
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|- | |- | ||
| style="background:#DCDCDC;" align="center" + |[[Anaplastic]] RMS | | style="background:#DCDCDC;" align="center" + |[[Anaplastic]] RMS | ||
| style="background:#F5F5F5;" +| | | style="background:#F5F5F5;" + | | ||
* Mostly seen in patients with [[Li-Fraumeni syndrome]]<ref name="pmid24382691">{{cite journal| author=Hettmer S, Archer NM, Somers GR, Novokmet A, Wagers AJ, Diller L et al.| title=Anaplastic rhabdomyosarcoma in TP53 germline mutation carriers. | journal=Cancer | year= 2014 | volume= 120 | issue= 7 | pages= 1068-75 | pmid=24382691 | doi=10.1002/cncr.28507 | pmc=4173134 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24382691 }}</ref> | * Mostly seen in patients with [[Li-Fraumeni syndrome]]<ref name="pmid24382691">{{cite journal| author=Hettmer S, Archer NM, Somers GR, Novokmet A, Wagers AJ, Diller L et al.| title=Anaplastic rhabdomyosarcoma in TP53 germline mutation carriers. | journal=Cancer | year= 2014 | volume= 120 | issue= 7 | pages= 1068-75 | pmid=24382691 | doi=10.1002/cncr.28507 | pmc=4173134 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24382691 }}</ref> | ||
* Contain 1% of all cases | * Contain 1% of all cases | ||
Latest revision as of 14:40, 13 March 2019
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Rhabdomyosarcoma Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
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Rhabdomyosarcoma classification On the Web |
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American Roentgen Ray Society Images of Rhabdomyosarcoma classification |
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Risk calculators and risk factors for Rhabdomyosarcoma classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Shadan Mehraban, M.D.[2]
Overview
The international classification of rhabdomyosarcoma (RMS) was created by the Intergroup Rhabdomyosarcoma Study group (IRSG). The four main subgroups include: Embryonal rhabdomyosarcoma (ERMS), Alveolar rhabdomyosarcoma (ARMS), Botryoid and spindle cell (leiomyomatous) RMS, Sclerosing and spindle cell RMS. The other separate categories for subtypes which do not classify into above groups are Undifferentiated, Pleomorphic/anaplastic, and Sarcoma, not otherwise specified (NOS).
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 rhabdomyosarcoma (ERMS)
- Alveolar rhabdomyosarcoma (ARMS)
- Botryoid and spindle cell (leiomyomatous) RMS
- Sclerosing and spindle cell RMS
- Separate categories for other subtypes which do not classify into above groups:[3]
- Undifferentiated
- Pleomorphic/anaplastic
- Sarcoma, not otherwise specified (NOS)
| RMS subtypes | Definition |
|---|---|
| Embryonal rhabdomyosarcoma (ERMS) |
|
| Alveolar rhabdomyosarcoma (ARMS) |
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| Botryoid and spindle cell RMS |
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| Sclerosing RMS |
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| Anaplastic RMS |
|
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.
- ↑ Kodet R, Newton WA, Hamoudi AB, Asmar L, Jacobs DL, Maurer HM (1993). "Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study". Am J Surg Pathol. 17 (5): 443–53. PMID 8470759.
- ↑ De Giovanni C, Landuzzi L, Nicoletti G, Lollini PL, Nanni P (2009). "Molecular and cellular biology of rhabdomyosarcoma". Future Oncol. 5 (9): 1449–75. doi:10.2217/fon.09.97. PMID 19903072.
- ↑ Scrable HJ, Witte DP, Lampkin BC, Cavenee WK (1987). "Chromosomal localization of the human rhabdomyosarcoma locus by mitotic recombination mapping". Nature. 329 (6140): 645–7. doi:10.1038/329645a0. PMID 3657988.
- ↑ Helman LJ, Meltzer P (2003). "Mechanisms of sarcoma development". Nat Rev Cancer. 3 (9): 685–94. doi:10.1038/nrc1168. PMID 12951587.
- ↑ Hettmer S, Archer NM, Somers GR, Novokmet A, Wagers AJ, Diller L; et al. (2014). "Anaplastic rhabdomyosarcoma in TP53 germline mutation carriers". Cancer. 120 (7): 1068–75. doi:10.1002/cncr.28507. PMC 4173134. PMID 24382691.