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Difference between revisions of "Leiomyosarcoma"
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = Cutaneous_leiomyosarcoma_- | | Image = Cutaneous_leiomyosarcoma_-_high_mag.jpg | ||
| Width = | | Width = | ||
| Caption = Leiomyosarcoma. [[H&E stain]]. | | Caption = Leiomyosarcoma. [[H&E stain]]. | ||
| Micro = | | 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 = | | 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 = | | 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 = | | Stains = | ||
| IHC = | | IHC = | ||
| Line 12: | Line 12: | ||
| Molecular = | | Molecular = | ||
| IF = | | IF = | ||
| Gross = | | Gross = "fleshy" appearance, necrosis, large size | ||
| Grossing = | | Grossing = | ||
| Site = [[uterus]], [[skin]] | | Site = [[uterus]], [[skin]], others | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = [[hereditary leiomyomatosis and renal cell cancer]] | ||
| Clinicalhx = | | Clinicalhx = | ||
| Signs = | | Signs = | ||
| Line 31: | Line 31: | ||
==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 [[leiomyoma]]s (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== | ||
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*#**Zonal necrosis is suggestive of vascular cause. | *#**Zonal necrosis is suggestive of vascular cause. | ||
*#Mitoses - '''key feature''' - definitions suffer from [[HPFitis]]: | *#Mitoses - '''key feature''' - definitions suffer from [[HPFitis]]: | ||
*#*>=10 mitoses/HPF - if spindled.<ref name=Ref_GP281>{{Ref GP|281}}</ref> | *#*>=10 mitoses/10 HPF - if spindled.<ref name=Ref_GP281>{{Ref GP|281}}</ref> | ||
*#*>=5 mitoses/HPF - if epithelioid.<ref name=Ref_GP281>{{Ref GP|281}}</ref> | *#*>=5 mitoses/10 HPF - if epithelioid.<ref name=Ref_GP281>{{Ref GP|281}}</ref> | ||
*#*>=2 mitoses/HPF - if [[myxoid]].<ref name=Ref_GP281>{{Ref GP|281}}</ref> | *#*>=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> | *+/-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> | ||
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*[[Smooth muscle tumour of uncertain malignant potential]] (STUMP). | *[[Smooth muscle tumour of uncertain malignant potential]] (STUMP). | ||
*[[Endometrial stromal sarcoma]]. | *[[Endometrial stromal sarcoma]]. | ||
*[[Atypical leiomyoma]]. | |||
===Images=== | ===Images=== | ||
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Image:Cutaneous_leiomyosarcoma_-_a_-_intermed_mag.jpg | Cutaneous leiomyosarcoma - intermed. mag. (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_-_high_mag.jpg | Cutaneous leiomyosarcoma - high mag. (WC) | ||
Image:Cutaneous_leiomyosarcoma_-_very_high_mag.jpg | Cutaneous leiomyosarcoma - very high mag. (WC) | |||
</gallery> | </gallery> | ||
www: | www: | ||
*[http://path.upmc.edu/cases/case107/micro.html Cutaneous leiomyosarcoma (upmc.edu)]. | *[http://path.upmc.edu/cases/case107/micro.html Cutaneous leiomyosarcoma (upmc.edu)]. | ||
===Subtypes=== | ===Subtypes=== | ||
Major variants:<ref name=Ref_GP284>{{Ref GP|284}}</ref> | Major variants:<ref name=Ref_GP284>{{Ref GP|284}}</ref> | ||
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*MIB1 high. | *MIB1 high. | ||
*Kertins usu. -ve -- more often +ve in epithelioid variant. | *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== | ||