Difference between revisions of "Leiomyosarcoma"

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Leiomyosarcoma (view source)

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Cutaneous_leiomyosarcoma_-_high_mag.jpg
| Width      =
| Caption    = Leiomyosarcoma. [[H&E stain]].
| Micro      = fasciclar arrangement (characteristic of smooth muscle), features of malignancy (usually need 2 of 3): † (1) nuclear atypia, (2) tumour cell necrosis, (3) mitoses - variable & definitions suffer from [[HPFitis]]
| Subtypes  = major: spindled leiomyosarcoma (leiomyosarcoma NOS), epithelioid leiomyosarcoma, myxoid leiomyosarcoma; minor: leiomyosarcoma with prominent intravascular growth, leiomyosarcoma with osteoclast-type cells, leiomyosarcoma with clear cells, leiomyosarcoma with xanthoma-type cells
| LMDDx      = [[pleomorphic undifferentiated sarcoma]], [[atypical fibroxanthoma]] (skin only), [[EBV-associated smooth muscle tumour]], [[carcinosarcoma]], [[smooth muscle tumour of uncertain malignant potential]] (STUMP), [[endometrial stromal sarcoma]], [[atypical leiomyoma]] (symplastic leiomyoma)
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      = "fleshy" appearance, necrosis, large size
| Grossing  =
| Site      = [[uterus]], [[skin]], others
| Assdx      =
| Syndromes  = [[hereditary leiomyomatosis and renal cell cancer]]
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    =
}}
'''Leiomyosarcoma''' is a malignant tumour of smooth muscle.  It is seen in various places including the [[uterus]] and [[skin]].
'''Leiomyosarcoma''' is a malignant tumour of smooth muscle.  It is seen in various places including the [[uterus]] and [[skin]].


==General==
==General==
*Poor prognosis.
*Poor prognosis - usually.
**In locally advanced/metastatic disease (arising from the uterus) the median survival is 12-14 months.<ref>{{Cite journal  | last1 = Hadoux | first1 = J. | last2 = Morice | first2 = P. | last3 = Lhommé | first3 = C. | last4 = Duvillard | first4 = P. | last5 = Balleyguier | first5 = C. | last6 = Haie-Meder | first6 = C. | last7 = Gouy | first7 = S. | last8 = Uzan | first8 = C. | last9 = Mazeron | first9 = R. | title = [Uterine leiomyosarcoma: epidemiology, pathology, biology, diagnosis, prognosis and treatment]. | journal = Bull Cancer | volume = 100 | issue = 9 | pages = 903-15 | month = Sep | year = 2013 | doi = 10.1684/bdc.2013.1801 | PMID = 24004576 }}</ref>
*Do not (generally) arise from leiomyomas.
*Do not (generally) arise from leiomyomas.
*Often singular, i.e. one tumour; unlike leiomyomas (which are often multiple).
*Often singular, i.e. one tumour; unlike [[leiomyoma]]s (which are often multiple).
*May be a part of [[hereditary leiomyomatosis and renal cell cancer]].
 
Note:
*''Skin leiomyosarcoma'' do so well it has been proposed to no longer refer to them as ''sarcomas''.<ref name=pmid21358302/>


==Gross==
==Gross==
Line 15: Line 49:
==Microscopic==
==Microscopic==
Features:
Features:
*Cellular lesion.
*Usually a cellular lesion.
*Fasicular arrangement:  
*Fasciclar arrangement:  
**Whorled look at low power.
**Whorled look at low power.
**Groups of spindle cells cut peripendicular to their long axis adjacent to groups of spindle cells cut in the plane of their long axis.
**Groups of spindle cells cut peripendicular to their long axis adjacent to groups of spindle cells cut in the plane of their long axis.
*Features of malignancy (need all three):
*Features of malignancy (usually need 2 of 3): †
*#Cellular atypia - common.
*#Nuclear atypia.
*#Necrosis.
*#Tumour cell necrosis.
*#*Should be patchy/multifocal.
*#*Should be patchy/multifocal.
*#**Zonal necrosis is suggestive of vascular cause.
*#**Zonal necrosis is suggestive of vascular cause.
*#Mitoses - '''key feature'''.
*#Mitoses - '''key feature''' - definitions suffer from [[HPFitis]]:
*#*10 mitoses/HPF.
*#*>=10 mitoses/10 HPF - if spindled.<ref name=Ref_GP281>{{Ref GP|281}}</ref>
*#*5 mitoses/HPF - if epithelioid.
*#*>=5 mitoses/10 HPF - if epithelioid.<ref name=Ref_GP281>{{Ref GP|281}}</ref>
*#*2 mitoses/HPF - if myxoid.
*#*>=2 mitoses/10 HPF - if [[myxoid]].<ref name=Ref_GP281>{{Ref GP|281}}</ref>
*#*>=1 mitosis/HPF - if cutaneous.<ref name=pmid21358302>{{cite journal |author=Kraft S, Fletcher CD |title=Atypical intradermal smooth muscle neoplasms: clinicopathologic analysis of 84 cases and a reappraisal of cutaneous "leiomyosarcoma" |journal=Am. J. Surg. Pathol. |volume=35 |issue=4 |pages=599–607 |year=2011 |month=April |pmid=21358302 |doi=10.1097/PAS.0b013e31820e6093 |url=}}</ref>
*#*>=1 mitosis/10 HPF - if cutaneous.<ref name=pmid21358302>{{cite journal |author=Kraft S, Fletcher CD |title=Atypical intradermal smooth muscle neoplasms: clinicopathologic analysis of 84 cases and a reappraisal of cutaneous "leiomyosarcoma" |journal=Am. J. Surg. Pathol. |volume=35 |issue=4 |pages=599–607 |year=2011 |month=April |pmid=21358302 |doi=10.1097/PAS.0b013e31820e6093 |url=}}</ref>
*+/-Heterologous elements, e.g. malignant cartilage or bone.<ref name=pmid22833086>{{Cite journal  | last1 = Anh Tran | first1 = T. | last2 = Holloway | first2 = RW. | title = Metastatic leiomyosarcoma of the uterus with heterologous differentiation to malignant mesenchymoma. | journal = Int J Gynecol Pathol | volume = 31 | issue = 5 | pages = 453-7 | month = Sep | year = 2012 | doi = 10.1097/PGP.0b013e318246977d | PMID = 22833086 }}</ref>
 
Notes:
*† In deep soft tissue. 1 of 3 criteria is considered enough.<ref>URL: [http://surgpathcriteria.stanford.edu/softsmoothmuscle/soft_tissue_leiomyosarcoma/differentialdiagnosis.html http://surgpathcriteria.stanford.edu/softsmoothmuscle/soft_tissue_leiomyosarcoma/differentialdiagnosis.html]. Accessed on: 10 May 2013.</ref>
*Leiomyosarcoma ''de facto'' trumps other sarcomas.<ref name=pmid22833086/>
*Mitotic rate seems to be a relatively weak predictor; modest rate may be malignant and a high rate benign.<ref name=pmid9388868>{{Cite journal  | last1 = Guo | first1 = L. | last2 = Liu | first2 = T. | last3 = Huang | first3 = H. | title = [Reappraisal of the pathological criteria for uterine leiomyosarcoma]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 25 | issue = 5 | pages = 266-9 | month = Oct | year = 1996 | doi =  | PMID = 9388868 }}</ref>
 
DDx:
*[[Pleomorphic undifferentiated sarcoma]].
*[[EBV-associated smooth muscle tumour]] - rare, immunoincompetent individuals.
*[[Carcinosarcoma]].
*[[Smooth muscle tumour of uncertain malignant potential]] (STUMP).
*[[Endometrial stromal sarcoma]].
*[[Atypical leiomyoma]].
 
===Images===
Uterine:
<gallery>
Image:Uterine_leiomyosarcoma_%281%29.jpg | Uterine leiomyosarcoma 1 (WC)
Image:Uterine_leiomyosarcoma_%282%29.jpg | Uterine leiomyosarcoma 2 (WC)
</gallery>
Cutaneous:
<gallery>
Image:Cutaneous_leiomyosarcoma_-_intermed_mag.jpg | Cutaneous leiomyosarcoma - intermed. mag. - shows fascicular pattern (WC)
Image:Cutaneous_leiomyosarcoma_-_a_-_intermed_mag.jpg | Cutaneous leiomyosarcoma - intermed. mag. (WC)
Image:Cutaneous_leiomyosarcoma_-_high_mag.jpg | Cutaneous leiomyosarcoma - high mag. (WC)
Image:Cutaneous_leiomyosarcoma_-_very_high_mag.jpg | Cutaneous leiomyosarcoma - very high mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case107/micro.html Cutaneous leiomyosarcoma (upmc.edu)].
 
===Subtypes===
Major variants:<ref name=Ref_GP284>{{Ref GP|284}}</ref>
*Spindled leiomyosarcoma (leiomyosarcoma NOS) - see above.
*Epithelioid leiomyosarcoma.
*Myxoid leiomyosarcoma.
 
Minor variants:<ref name=Ref_GP284>{{Ref GP|284}}</ref>
*Leiomyosarcoma with prominent intravascular growth.
*Leiomyosarcoma with osteoclast-type cells.
*Leiomyosarcoma with clear cells.
*Leiomyosarcoma with xanthoma-type cells.
 
====Epithelioid leiomyosarcoma====
Features:<ref name=Ref_GP281>{{Ref GP|281}}</ref>
*>50% epithelial appearance.
*>=5 mitoses/HPF - definition suffers from [[HPFitis]].
 
Image:
*[http://www.webpathology.com/image.asp?n=12&Case=61 Epithelioid leiomyosarcoma (webpathology.com)].
 
====Myxoid leiomyosarcoma====
Features:<ref name=Ref_GP281>{{Ref GP|281}}</ref>
*>=2 mitoses/HPF - definition suffers from [[HPFitis]].
*May have minimal nuclear atypia.


===IHC===
==IHC==
*CD10 -ve (important in the context of uterine lesions -- +ve in [[endometrial stromal sarcoma]]).
Features:
*Positive for SMC markers.
*Positive for SMC markers.
**Desmin - present in all three types of muscle.
**Desmin - present in all three types of muscle.
**Caldesmon.
**H-caldesmon.
**Smooth muscle myosin.
**Smooth muscle myosin.
*CD10 -ve.
**May be +ve.<ref name=Ref_GP284>{{Ref GP|284}}</ref>
**Some use in the context of uterine lesions -- CD10 +ve in [[endometrial stromal sarcoma]].
Others:<ref name=Ref_GP284>{{Ref GP|284}}</ref>
*ER, PR, AR +ve -- 30-40% of the time.
*CD117 +ve/-ve.
*p53 +ve.
*MIB1 high.
*Kertins usu. -ve -- more often +ve in epithelioid variant.
==Sign out==
<pre>
SKIN LESION, LEFT SHOULDER (SUTURE AT 12 O'CLOCK), WIDE EXCISION:
- LEIOMYOSARCOMA, MARGINALLY EXCISED.
-- PLEASE SEE TUMOUR SUMMARY.
- DERMAL SCAR.
</pre>


==See also==
==See also==
*[[Soft tissue lesions]].
*[[Soft tissue lesions]].
*[[Uterine tumours]].
*[[Uterine tumours]].
*[[Leiomyoma]].


==References==
==References==
{{Reflist|1}}
{{Reflist|2}}


[[Category:Soft tissue]]
[[Category:Soft tissue lesions]]
[[Category:Gynecologic pathology]]
[[Category:Gynecologic pathology]]
Michael
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