Rhabdomyosarcoma classification: Difference between revisions
Jump to navigation
Jump to search
No edit summary |
|||
| (5 intermediate revisions by the same user not shown) | |||
| Line 3: | Line 3: | ||
{{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 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== | ||
| Line 8: | Line 9: | ||
*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 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: | *The four main subgroups are: | ||
**Embryonal rhabdomyosarcoma (ERMS) | **[[Embryonal rhabdomyosarcoma]] (ERMS) | ||
**Alveolar rhabdomyosarcoma (ARMS) | **[[Alveolar rhabdomyosarcoma]] (ARMS) | ||
**Botryoid and spindle cell (leiomyomatous) RMS | **[[Botryoid rhabdomyosarcoma|Botryoid]] and [[spindle cell]] (leiomyomatous) RMS | ||
**Sclerosing and spindle cell RMS | **Sclerosing and [[spindle cell]] RMS | ||
*Separate categories for other subtypes which do not classify into above groups: | *Separate categories for other subtypes which do not classify into above groups:<ref name="pmid8470759">{{cite journal| author=Kodet R, Newton WA, Hamoudi AB, Asmar L, Jacobs DL, Maurer HM| title=Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study. | journal=Am J Surg Pathol | year= 1993 | volume= 17 | issue= 5 | pages= 443-53 | pmid=8470759 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8470759 }}</ref> | ||
**Undifferentiated | **[[Undifferentiated]] | ||
**Pleomorphic/anaplastic | **[[Pleomorphic]]/[[anaplastic]] | ||
**Sarcoma | **[[Sarcoma]], not otherwise specified (NOS) | ||
{| class="wikitable" | {| class="wikitable" | ||
|+ | |+ | ||
!RMS subtypes | ! style="background:#4479BA; color: #FFFFFF;" align="center" + |RMS subtypes | ||
!Definition | ! style="background:#4479BA; color: #FFFFFF;" align="center" + |Definition | ||
|- | |- | ||
|Embryonal | | style="background:#DCDCDC;" align="center" + |[[Embryonal rhabdomyosarcoma]] (ERMS) | ||
| | | style="background:#F5F5F5;" + | | ||
* The most common group | * The most common group | ||
* Contains 59% of all RMS | * Contains 59% of all RMS | ||
* Intermediate prognosis | * 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> | * 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 | * 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> | * 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 | * [[Loss of heterozygosity]] (LOH) at the 11p15 [[locus]], the site of the IGF-2 [[gene]] | ||
|- | |- | ||
|Alveolar | | style="background:#DCDCDC;" align="center" + |[[Alveolar rhabdomyosarcoma]] (ARMS) | ||
| | | 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]] | ||
* Contains 21% of all RMS | * Contains 21% of all RMS | ||
* More likely among adolescents | * More likely among adolescents | ||
* Mostly occur in the trunk and extremities | * Mostly occur in the [[trunk]] and [[extremities]] | ||
|- | |- | ||
|Botryoid and spindle cell RMS | | style="background:#DCDCDC;" align="center" + |[[Botryoid rhabdomyosarcoma|Botryoid]] and [[spindle cell]] RMS | ||
| | | style="background:#F5F5F5;" + | | ||
* 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 | ||
* Less common | * Less common variants of ERMS | ||
* Accompanied with more favorable prognosis | * Accompanied with more favorable [[prognosis]] | ||
* Botryoid name related to grape-like appearance of tumor | * [[Botryoid rhabdomyosarcoma|Botryoid]] name related to grape-like appearance of [[tumor]] | ||
* Botryoid RMS occur within bladder wall and vagina | * [[Botryoid rhabdomyosarcoma|Botryoid]] RMS occur within [[bladder]] wall and [[vagina]] | ||
* Spindle cell is prevalent among infants and neonates under age of 1 years old | * [[Spindle cell]] is prevalent among infants and neonates under age of 1 years old | ||
* Spindle cell RMS occurs in paratesticular location | * [[Spindle cell]] RMS occurs in paratesticular location | ||
|- | |- | ||
|Sclerosing RMS | | style="background:#DCDCDC;" align="center" + |Sclerosing RMS | ||
| | | style="background:#F5F5F5;" + | | ||
* Less common variants of ERMS | * Less common variants of ERMS | ||
* Poor outcome | * Poor outcome | ||
* Mostly occurs after age of 1 years old | * Mostly occurs after age of 1 years old | ||
* Could also be seen among adults | * Could also be seen among adults | ||
* Accompanied with MyoD1 mutation | * Accompanied with MyoD1 [[mutation]] | ||
* | * | ||
|- | |- | ||
|Anaplastic RMS | | style="background:#DCDCDC;" align="center" + |[[Anaplastic]] RMS | ||
| | | 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 | ||
* Defined as a large hyperchromatic nuclei | * Defined as a large hyperchromatic [[nuclei]] | ||
|} | |} | ||
Latest revision as of 14:40, 13 March 2019
|
Rhabdomyosarcoma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Rhabdomyosarcoma classification On the Web |
|
American Roentgen Ray Society Images of Rhabdomyosarcoma classification |
|
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) |
|
| Botryoid and spindle cell RMS |
|
| Sclerosing RMS |
|
| 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.