Leiomyosarcoma: Difference between revisions
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| Site = [[uterus]], [[skin]], others | | Site = [[uterus]], [[skin]], others | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = [[hereditary leiomyomatosis and renal cell cancer]] | ||
| Clinicalhx = | | Clinicalhx = | ||
| Signs = | | Signs = | ||
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==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> | ||