Rhabdomyosarcoma: Difference between revisions
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = Alveolar_rhabdomyosarcoma_-_very_high_mag.jpg | | Image = Alveolar_rhabdomyosarcoma_-_very_high_mag.jpg | ||
| Width = | | Width = | ||
| Caption = Alveolar rhabdomyosarcoma. [[H&E stain]]. | | Caption = Alveolar rhabdomyosarcoma. [[H&E stain]]. | ||
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| Site = [[soft tissue]] - skeletal muscle site (alveolar RMS), non-skeletal muscle site (embryonal RMS) | | Site = [[soft tissue]] - skeletal muscle site (alveolar RMS), non-skeletal muscle site (embryonal RMS) | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = [[DICER1 syndrome]] for ''embryonal rhabdomyosarcoma'' | ||
| Clinicalhx = alveolar RMS: young adult or adolescent; embryonal RMS: typically <10 years old | | Clinicalhx = alveolar RMS: young adult or adolescent; embryonal RMS: typically <10 years old | ||
| Signs = | | Signs = | ||
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==Microscopic== | ==Microscopic== | ||
===Alveolar rhabdomyosarcoma=== | ===Alveolar rhabdomyosarcoma=== | ||
Features:<ref name=PST14feb11 | Features:<ref name=PST14feb11/> | ||
*Alveolus-like pattern -- '''key low-power feature'''. | *Alveolus-like pattern -- '''key low-power feature'''. | ||
**Fibrous septae lined by tumour cells. | **Fibrous septae lined by tumour cells. | ||
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===Embryonal rhabdomyosarcoma=== | ===Embryonal rhabdomyosarcoma=== | ||
Features:<ref name=PST14feb11 | Features:<ref name=PST14feb11/> | ||
*Randomly arranged small cells. | *Randomly arranged small cells. | ||
*[[Myxoid]] matrix. | *[[Myxoid]] matrix. | ||
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===Subtyping via IHC=== | ===Subtyping via IHC=== | ||
PST proposes<ref name=PST14feb11 | PST proposes<ref name=PST14feb11/> the following (presumably based on Makawitz et al.<ref name=pmid18788888>{{cite journal |author=Makawita S, Ho M, Durbin AD, Thorner PS, Malkin D, Somers GR |title=Expression of insulin-like growth factor pathway proteins in rhabdomyosarcoma: IGF-2 expression is associated with translocation-negative tumors |journal=Pediatr. Dev. Pathol. |volume=12 |issue=2 |pages=127–35 |year=2009 |pmid=18788888 |doi=10.2350/08-05-0477.1 |url=}}</ref>): | ||
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto" | {| class="wikitable sortable" style="margin-left:auto;margin-right:auto" | ||
| '''IHC''' | | '''IHC''' | ||
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*t(1,13) vs. t(2,13) -- t(1,13) usually: younger age, extremity lesion, localized disease, better survival. | *t(1,13) vs. t(2,13) -- t(1,13) usually: younger age, extremity lesion, localized disease, better survival. | ||
*Several uncommon [[translocations]] exist. | *Several uncommon [[translocations]] exist. | ||
*'''Important''' for [[urinary bladder]] lesions in adults: the presence of a translocation is more-or-less required for the diagnosis of RMS.<ref name=pmid21762516>{{Cite journal | last1 = Bing | first1 = Z. | last2 = Zhang | first2 = PJ. | title = Adult urinary bladder tumors with rhabdomyosarcomatous differentiation: clinical, pathological and immunohistochemical studies. | journal = Diagn Pathol | volume = 6 | issue = | pages = 66 | month = | year = 2011 | doi = 10.1186/1746-1596-6-66 | PMID = 21762516 }}</ref> | |||
**It is suggested that keratin negative tumours without molecular testing to corroborate the impression of RMS be referred to as ''rhabdomyomatous tumours''.<ref name=pmid21762516/> | |||
===Embryonal rhabdomyosarcoma=== | ===Embryonal rhabdomyosarcoma=== | ||