Rhabdomyosarcoma classification: Difference between revisions

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Revision as of 16:58, 11 January 2019

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 rhabdomyosarcoma (ERMS)
- Alveolar rhabdomyosarcoma (ARMS)
- Botryoid and spindle cell (leiomyomatous) RMS
- Sclerosing and spindle cell RMS


RMS subtypes	Definition
Embryonal RMS (ERMS)	The most common group Contains 59% of all RMS Intermediate prognosis Mostly occurs in head, neck, and genitourinary system^[3] Larger peak between age of 0-5 years and smaller peak among adolescents Specific translocation is not yet designated^[4] Loss of heterozygosity (LOH) at the 11p15 locus, the site of the IGF-2 gene
Alveolar RMS (ARMS)	Determined by FOXO1 rearrangement, t (1;13) or t (2;13)^[5] Poorer prognosis Contains 21% of all RMS More likely among adolescents Mostly occur in the trunk and extremities

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.
↑ 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.

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[pmid9724344-1] 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.

[pmid12110339-2] Hicks J, Flaitz C (2002). "Rhabdomyosarcoma of the head and neck in children". Oral Oncol. 38 (5): 450–9. PMID 12110339.

[pmid19903072-3] 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.

[pmid3657988-4] 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.

[pmid12951587-5] Helman LJ, Meltzer P (2003). "Mechanisms of sarcoma development". Nat Rev Cancer. 3 (9): 685–94. doi:10.1038/nrc1168. PMID 12951587.

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@@ Line 8: / Line 8: @@
 *The international classification of rhabdomyosarcoma (RMS) was created by the Intergroup Rhabdomyosarcoma Study group (IRSG) therapeutic trials (IRS-I to IV).<ref name="pmid9724344">{{cite journal| author=Qualman SJ, Coffin CM, Newton WA, Hojo H, Triche TJ, Parham DM et al.| title=Intergroup Rhabdomyosarcoma Study: update for pathologists. | journal=Pediatr Dev Pathol | year= 1998 | volume= 1 | issue= 6 | pages= 550-61 | pmid=9724344 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9724344  }} </ref><ref name="pmid12110339">{{cite journal| author=Hicks J, Flaitz C| title=Rhabdomyosarcoma of the head and neck in children. | journal=Oral Oncol | year= 2002 | volume= 38 | issue= 5 | pages= 450-9 | pmid=12110339 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12110339  }} </ref>
 *The four main subgroups are:
-**Embryonal RMS
+**Embryonal rhabdomyosarcoma (ERMS)
+**Alveolar rhabdomyosarcoma (ARMS)
 **Botryoid and spindle cell (leiomyomatous) RMS
-**Sclerosing and spindle cell RMS with MyoD1 mutation
+**Sclerosing and spindle cell RMS
-**Alveolar RMS
+{| class="wikitable"
+|+
+!RMS subtypes
+!Definition
+|-
+|Embryonal RMS (ERMS)
+|
+* The most common group
+* Contains 59% of all RMS
+* Intermediate prognosis
+* Mostly occurs in head, neck, and genitourinary system<ref name="pmid19903072">{{cite journal| author=De Giovanni C, Landuzzi L, Nicoletti G, Lollini PL, Nanni P| title=Molecular and cellular biology of rhabdomyosarcoma. | journal=Future Oncol | year= 2009 | volume= 5 | issue= 9 | pages= 1449-75 | pmid=19903072 | doi=10.2217/fon.09.97 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19903072  }}</ref>
+* Larger peak between age of 0-5  years and smaller peak among adolescents
+* Specific translocation is not yet designated<ref name="pmid3657988">{{cite journal| author=Scrable HJ, Witte DP, Lampkin BC, Cavenee WK| title=Chromosomal localization of the human rhabdomyosarcoma locus by mitotic recombination mapping. | journal=Nature | year= 1987 | volume= 329 | issue= 6140 | pages= 645-7 | pmid=3657988 | doi=10.1038/329645a0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3657988  }}</ref>
+* Loss of heterozygosity (LOH) at the 11p15 locus, the site of the IGF-2 gene
+|-
+|Alveolar RMS (ARMS)
+|
+* 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
+* Contains 21% of all RMS
+* More likely among adolescents
+* Mostly occur in the trunk and extremities
+|-
+|
+|
+|}
 *Alveolar rhabdomyosarcoma
 **Usually affects young adults