Difference between revisions of "Endometrial stromal sarcoma"

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Endometrial stromal sarcoma (view source)

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#redirect [[Uterine_tumours#Endometrial stromal sarcoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Endometrial stromal sarcoma - low mag.jpg
| Width      =
| Caption    = Endometrial stromal sarcoma. [[H&E stain]].
| Micro      = highly cellular islands of (tumour) cells with a wavy irregular border -- benign uterine smooth muscle between; tumour cells: high [[NC ratio]] and epithelioid, pallisade around thin blood vessels within the tumour
| Subtypes  =
| LMDDx      = [[leiomyoma]] (cellular), [[endometrial stromal nodule]], [[uterine leiomyosarcoma]], [[uterine tumour resembling an ovarian sex cord stromal tumour]]
| Stains    =
| IHC        = [[CD10]] +ve, H-caldesmin -ve, PR +/-ve, ER +/-ve
| EM        =
| Molecular  = t(7;17)
| IF        =
| Gross      =
| Grossing  =
| Site      = [[uterus]] - see [[uterine tumours]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good
| Other      =
| ClinDDx    = other [[uterine tumours]]
}}
'''Endometrial stromal sarcoma''', abbreviated '''ESS''', is a rare malignant mesenchymal [[uterine tumours|tumour of the uterus]].


It is also known as '''low-grade endometrial stromal sarcoma''', abbreviated '''LGESS'''.
==General==
*Uncommon.<ref name=pmid20179433> {{Cite journal  | last1 = Chew | first1 = I. | last2 = Oliva | first2 = E. | title = Endometrial stromal sarcomas: a review of potential prognostic factors. | journal = Adv Anat Pathol | volume = 17 | issue = 2 | pages = 113-21 | month = Mar | year = 2010 | doi = 10.1097/PAP.0b013e3181cfb7c2 | PMID = 20179433 }}</ref>
*Prognosis good<ref name=pmid24146786>{{Cite journal  | last1 = Feng | first1 = W. | last2 = Malpica | first2 = A. | last3 = Skaland | first3 = I. | last4 = Gudlaugsson | first4 = E. | last5 = Robboy | first5 = SJ. | last6 = Dalen | first6 = I. | last7 = Hua | first7 = K. | last8 = Zhou | first8 = X. | last9 = Baak | first9 = JP. | title = Can proliferation biomarkers reliably predict recurrence in world health organization 2003 defined endometrial stromal sarcoma, low grade? | journal = PLoS One | volume = 8 | issue = 10 | pages = e75899 | month =  | year = 2013 | doi = 10.1371/journal.pone.0075899 | PMID = 24146786 }}</ref> > 90% five year survival and > 85% ten year survival.<ref name=pmid24184602>{{Cite journal  | last1 = Yoon | first1 = A. | last2 = Park | first2 = JY. | last3 = Park | first3 = JY. | last4 = Lee | first4 = YY. | last5 = Kim | first5 = TJ. | last6 = Choi | first6 = CH. | last7 = Bae | first7 = DS. | last8 = Kim | first8 = BG. | last9 = Lee | first9 = JW. | title = Prognostic factors and outcomes in endometrial stromal sarcoma with the 2009 FIGO staging system: A multicenter review of 114 cases. | journal = Gynecol Oncol | volume =  | issue =  | pages =  | month = Oct | year = 2013 | doi = 10.1016/j.ygyno.2013.10.029 | PMID = 24184602 }}</ref>
Note:
*''Extrauterine endometrial stromal sarcoma'' is known as ''endometrioid stromal sarcoma''.<ref name=pmid24121169>{{Cite journal  | last1 = Masand | first1 = RP. | last2 = Euscher | first2 = ED. | last3 = Deavers | first3 = MT. | last4 = Malpica | first4 = A. | title = Endometrioid stromal sarcoma: a clinicopathologic study of 63 cases. | journal = Am J Surg Pathol | volume = 37 | issue = 11 | pages = 1635-47 | month = Nov | year = 2013 | doi = 10.1097/PAS.0000000000000083 | PMID = 24121169 }}</ref>
==Microscopic==
Features:
*Highly cellular islands with a wavy irregular border.
**Border has finger-like projections/[[tongue]]-like projections.
**Benign uterine smooth muscle between islands of tumour cells.
*Epithelioid cells.
*High [[NC ratio]].
*Thin blood vessels within islands of cells.
**Tumour cells pallisade around the vessels.
Notes:
*Vaguely resembles the stroma of proliferative endometrium.
*Low mitotic rate - predictive of good outcome.<ref name=pmid24146786/>
DDx:
*[[Leiomyoma]].
*[[Endometrial stromal nodule]].
*[[Uterine leiomyosarcoma]].
*[[Undifferentiated endometrial sarcoma]] (previously known as ''high-grade endometrial stromal sarcoma'') - necrosis and nuclear atypia.<ref name=pmid24146786/>
*[[Uterine tumour resembling an ovarian sex cord stromal tumour]].
===Images===
<gallery>
Image: Endometrial stromal sarcoma - low mag.jpg | ESS - low mag. (WC/Nephron)
Image: Endometrial stromal sarcoma - alt - low mag.jpg | ESS - low mag. (WC/Nephron)
Image: Endometrial stromal sarcoma - intermed mag.jpg | ESS - intermed. mag. (WC/Nephron)
Image: Endometrial stromal sarcoma - high mag.jpg | ESS - high mag. (WC/Nephron)
Image: Endometrial stromal sarcoma - very high mag.jpg | ESS - very high mag. (WC/Nephron)
</gallery>
<gallery>
Image:EndometrialStromalSarcoma.JPG | ESS. (WC)
Image:EndometrialStromalSarcomaLowGrade.JPG | Endometrial stromal sarcoma - low grade. (WC)
</gallery>
www:
*[http://jcp.bmj.com/content/60/3/235/F5.large.jpg ESS in an article with many crappy images].<ref name=pmid17347285>{{Cite journal  | last1 = Baker | first1 = P. | last2 = Oliva | first2 = E. | title = Endometrial stromal tumours of the uterus: a practical approach using conventional morphology and ancillary techniques. | journal = J Clin Pathol | volume = 60 | issue = 3 | pages = 235-43 | month = Mar | year = 2007 | doi = 10.1136/jcp.2005.031203 | PMID = 17347285 | url = http://jcp.bmj.com/content/60/3/235.full }}</ref>
*[http://radiographics.rsna.org/content/25/1/21/F42.expansion.html ESS (rsna.org)].
==IHC==
Features:<ref name=pmid14751141>{{Cite journal  | last1 = Zhu | first1 = XQ. | last2 = Shi | first2 = YF. | last3 = Cheng | first3 = XD. | last4 = Zhao | first4 = CL. | last5 = Wu | first5 = YZ. | title = Immunohistochemical markers in differential diagnosis of endometrial stromal sarcoma and cellular leiomyoma. | journal = Gynecol Oncol | volume = 92 | issue = 1 | pages = 71-9 | month = Jan | year = 2004 | doi =  | PMID = 14751141 }}</ref>
*[[CD10]] +ve.
*H-caldesmin -ve.
**[[Leiomyoma]]s +ve.
*PR +/-ve.
*ER +/-ve.
*CD117 +ve (~80%).<ref>{{Cite journal  | last1 = Park | first1 = JY. | last2 = Kim | first2 = KR. | last3 = Nam | first3 = JH. | title = Immunohistochemical analysis for therapeutic targets and prognostic markers in low-grade endometrial stromal sarcoma. | journal = Int J Gynecol Cancer | volume = 23 | issue = 1 | pages = 81-9 | month = Jan | year = 2013 | doi = 10.1097/IGC.0b013e3182738361 | PMID = 23221606 }}</ref>
==Molecular==
May be associated a recurrent [[translocation]]:<ref name=pmid12648605>{{Cite journal  | last1 = Amant | first1 = F. | last2 = Moerman | first2 = P. | last3 = Cadron | first3 = I. | last4 = Hagemeijer | first4 = A. | last5 = Vergote | first5 = I. | last6 = Debiec-Rychter | first6 = M. | title = Endometrial stromal sarcoma with a sole t(X;17) chromosome change: report of a case and review of the literature. | journal = Gynecol Oncol | volume = 88 | issue = 3 | pages = 459-62 | month = Mar | year = 2003 | doi =  | PMID = 12648605 | URL =  http://www.sciencedirect.com/science/article/pii/S0090825802000963 }}</ref>
*t(7;17)(p15;q21).
**JAZF1 - chromosome 7.<ref name=omim606246>{{OMIM|606246}}</ref>
**SUZ12 - chromosome 17.<ref name=omim606245>{{OMIM|606245}}</ref>
==See also==
*[[Uterine tumours]].
==References==
{{Reflist|2}}
[[Category:Gynecologic pathology]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]
Michael
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