Rhabdomyosarcoma - Revision history

Michael at 12:40, 24 March 2024

2024-03-24T12:40:03Z

← Older revision		Revision as of 12:40, 24 March 2024
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	==Microscopic==		==Microscopic==
	===Alveolar rhabdomyosarcoma===		===Alveolar rhabdomyosarcoma===
	Features:<ref name=PST14feb11~~>PST. 14 February 2011.<~~/~~ref~~>		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~~>PST. 14 February 2011.<~~/~~ref~~>		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. 14 February 2011.<~~/~~ref~~> 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>):		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'''

Michael at 12:36, 24 March 2024

2024-03-24T12:36:44Z

← Older revision		Revision as of 12:36, 24 March 2024
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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 =

Michael: touch

2015-03-03T03:34:47Z

touch

← Older revision		Revision as of 03:34, 3 March 2015
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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]].

Michael: /* Alveolar rhabdomyosarcoma */

2014-09-18T04:20:46Z

Alveolar rhabdomyosarcoma

← Older revision		Revision as of 04:20, 18 September 2014
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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 ~~the~~ [[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>		*'''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/>		**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/>

Michael: /* Molecular diagnostics */

2014-09-18T03:51:36Z

Molecular diagnostics

← Older revision		Revision as of 03:51, 18 September 2014
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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.
	*In the [[urinary bladder]] of 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>		*'''Important''' for the [[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/>		**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/>

Michael: /* Molecular diagnostics */

2014-09-18T03:49:25Z

Molecular diagnostics

← Older revision		Revision as of 03:49, 18 September 2014
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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.
			*In the [[urinary bladder]] of 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===

Michael: /* IHC */

2014-09-18T03:43:25Z

IHC

← Older revision		Revision as of 03:43, 18 September 2014
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	*Myogenin.		*Myogenin.

	~~Others~~:<ref name=pmid18487991>{{Cite journal \| last1 = Bahrami \| first1 = A. \| last2 = Gown \| first2 = AM. \| last3 = Baird \| first3 = GS. \| last4 = Hicks \| first4 = MJ. \| last5 = Folpe \| first5 = AL. \| title = Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall. \| journal = Mod Pathol \| volume = 21 \| issue = 7 \| pages = 795-806 \| month = Jul \| year = 2008 \| doi = 10.1038/modpathol.2008.86 \| PMID = 18487991 }}</ref>		For [[head and neck pathology\|head and neck]] RMS:<ref name=pmid18487991>{{Cite journal \| last1 = Bahrami \| first1 = A. \| last2 = Gown \| first2 = AM. \| last3 = Baird \| first3 = GS. \| last4 = Hicks \| first4 = MJ. \| last5 = Folpe \| first5 = AL. \| title = Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall. \| journal = Mod Pathol \| volume = 21 \| issue = 7 \| pages = 795-806 \| month = Jul \| year = 2008 \| doi = 10.1038/modpathol.2008.86 \| PMID = 18487991 }}</ref>
	*CD56 +ve.		*CD56 +ve.
	*Synaptophysin -ve/+ve (seen in 12 of 37 cases<ref name=pmid18487991/>).		*Synaptophysin -ve/+ve (seen in 12 of 37 cases<ref name=pmid18487991/>).
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	*Wide-spectrum cytokeratin -ve/+ve.		*Wide-spectrum cytokeratin -ve/+ve.
	*CAM5.2 -ve/+ve.		*CAM5.2 -ve/+ve.

			For [[urinary bladder]] RMS in adults:
			*Myogenin +ve.
			*Desmin +ve.
			*Keratins -ve.<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>
			**Keratin positive tumours are considered ''rhabdomyosarcomatous sarcomatoid carcinoma'' or ''sarcomatoid carcinoma with rhabdomyosarcomatous differentiation''.

	===Subtyping via IHC===		===Subtyping via IHC===

Michael at 04:29, 17 September 2014

2014-09-17T04:29:10Z

← Older revision		Revision as of 04:29, 17 September 2014
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	\| Gross =		\| Gross =
	\| Grossing =		\| Grossing =
	\| Site = [[soft tissue]] - skeletal muscle (alveolar RMS), non-muscle site (embryonal RMS)		\| Site = [[soft tissue]] - skeletal muscle site (alveolar RMS), non-skeletal muscle site (embryonal RMS)
	\| Assdx =		\| Assdx =
	\| Syndromes =		\| Syndromes =
	\| Clinicalhx =		\| Clinicalhx = alveolar RMS: young adult or adolescent; embryonal RMS: typically <10 years old
	\| Signs =		\| Signs =
	\| Symptoms =		\| Symptoms =
	\| Prevalence =		\| Prevalence = not common
	\| Bloodwork =		\| Bloodwork =
	\| Rads =		\| Rads =

Michael: +infobox

2014-09-17T04:27:01Z

+infobox

← Older revision		Revision as of 04:27, 17 September 2014
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			{{ Infobox diagnosis
			\| Name = {{PAGENAME}}
			\| Image = Alveolar_rhabdomyosarcoma_-_very_high_mag.jpg
			\| Width =
			\| Caption = Alveolar rhabdomyosarcoma. [[H&E stain]].
			\| Synonyms =
			\| Micro = +/-rhabdomyoblasts (eccentric nucleus, moderate amount of intensly eosinophilic cytoplasm, striations - not common); alveolar RMS: alveolus-like pattern (classic); embryonal RMS: [[small round cell tumour]]
			\| Subtypes = embryonal (spindle cell subtype, botryoid), alveolar (translocation-positive, translocation-negative), undifferentiated
			\| LMDDx = [[small round cell tumours]] - esp. [[small cell carcinoma]] and (large cell) [[lymphoma]]s
			\| Stains =
			\| IHC = desmin (best marker) +ve, actin +ve, myogenin +ve, CD56 +ve (common), synaptophysin -ve/+ve, chromogranin -ve/+ve, cytokeratins -ve/+ve
			\| EM = sarcomeric like structures - typically in U-shaped cells
			\| Molecular = alveolar RMS (~85% of cases): t(2,13) PAX3/FKHR fusion gene ''or'' t(1,13) PAX7/FKHR fusion gene
			\| IF =
			\| Gross =
			\| Grossing =
			\| Site = [[soft tissue]] - skeletal muscle (alveolar RMS), non-muscle site (embryonal RMS)
			\| Assdx =
			\| Syndromes =
			\| Clinicalhx =
			\| Signs =
			\| Symptoms =
			\| Prevalence =
			\| Bloodwork =
			\| Rads =
			\| Endoscopy =
			\| Prognosis =
			\| Other =
			\| ClinDDx = other soft tissue tumours
			\| Tx =
			}}
	'''Rhabdomyosarcoma''', often abbreviated '''RMS''', is a [[malignant]] tumour of skeletal muscle.		'''Rhabdomyosarcoma''', often abbreviated '''RMS''', is a [[malignant]] tumour of skeletal muscle.

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	#*Usually young adults/adolescents.		#*Usually young adults/adolescents.
	#*Early mets common.		#*Early mets common.
	#*~~Usu.~~ arises in regions with skeletal muscle.		#*Usually arises in regions with skeletal muscle.
	#Embryonal rhabdomyosarcoma.		#Embryonal rhabdomyosarcoma.
	#*Usual <10 years old.		#*Usual <10 years old.
	#*Typically locally invasive.		#*Typically locally invasive.
	#*~~Usu.~~ arises in regions '''without''' skeletal muscle.		#*Usually arises in regions '''without''' skeletal muscle.

	Less common types:<ref name=pmid12110339>{{Cite journal \| last1 = Hicks \| first1 = J. \| last2 = Flaitz \| first2 = C. \| title = Rhabdomyosarcoma of the head and neck in children. \| journal = Oral Oncol \| volume = 38 \| issue = 5 \| pages = 450-9 \| month = Jul \| year = 2002 \| doi = \| PMID = 12110339 }}</ref>		Less common types:<ref name=pmid12110339>{{Cite journal \| last1 = Hicks \| first1 = J. \| last2 = Flaitz \| first2 = C. \| title = Rhabdomyosarcoma of the head and neck in children. \| journal = Oral Oncol \| volume = 38 \| issue = 5 \| pages = 450-9 \| month = Jul \| year = 2002 \| doi = \| PMID = 12110339 }}</ref>
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	Other features:		Other features:
	*Nuclear pleomorphism - common.		*[[Nuclear pleomorphism]] - common.
	*Mitoses - common.		*Mitoses - common.

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	==[[Electron microscopy]]==		==[[Electron microscopy]]==
	Features:		Features:
	*Sarcomeric like structures - ~~usu.~~ in "bent" cells; cells that are U-shaped.		*Sarcomeric like structures - usually in "bent" cells; cells that are U-shaped.

	==Molecular diagnostics==		==Molecular diagnostics==

Michael: /* IHC */

2014-09-13T17:23:37Z

IHC

← Older revision		Revision as of 17:23, 13 September 2014
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	*Actin.		*Actin.
	*Myogenin.		*Myogenin.

			Others:<ref name=pmid18487991>{{Cite journal \| last1 = Bahrami \| first1 = A. \| last2 = Gown \| first2 = AM. \| last3 = Baird \| first3 = GS. \| last4 = Hicks \| first4 = MJ. \| last5 = Folpe \| first5 = AL. \| title = Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall. \| journal = Mod Pathol \| volume = 21 \| issue = 7 \| pages = 795-806 \| month = Jul \| year = 2008 \| doi = 10.1038/modpathol.2008.86 \| PMID = 18487991 }}</ref>
			*CD56 +ve.
			*Synaptophysin -ve/+ve (seen in 12 of 37 cases<ref name=pmid18487991/>).
			*Chromogranin A -ve/+ve (seen in 8 of 36 cases<ref name=pmid18487991/>).
			*Wide-spectrum cytokeratin -ve/+ve.
			*CAM5.2 -ve/+ve.

	===Subtyping via IHC===		===Subtyping via IHC===