Difference between revisions of "Endometrial stromal sarcoma"

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{{ 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]].
'''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'''
It is also known as '''low-grade endometrial stromal sarcoma''', abbreviated '''LGESS'''.


==General==
==General==
*Rare.<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>
*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==
==Microscopic==
Features:
Features:
*Highly cellular Islands with a wavy irregular border.
*Highly cellular islands with a wavy irregular border.
**Border has finger-like projections/[[tongue]]-like projections.
**Border has finger-like projections/[[tongue]]-like projections.
**Benign uterine smooth muscle between islands of tumour cells.
**Benign uterine smooth muscle between islands of tumour cells.
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Notes:
Notes:
*Vaguely resembles the stroma of proliferative endometrium.
*Vaguely resembles the stroma of proliferative endometrium.
*Low mitotic rate - predictive of good outcome.<ref name=pmid24146786/>


DDx:
DDx:
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*[[Endometrial stromal nodule]].
*[[Endometrial stromal nodule]].
*[[Uterine leiomyosarcoma]].
*[[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===
===Images===
*[http://commons.wikimedia.org/wiki/File:EndometrialStromalSarcoma.JPG ESS (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:EndometrialStromalSarcomaLowGrade.JPG Endometrial stromal sarcoma - low grade (WC)].
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://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)].
*[http://radiographics.rsna.org/content/25/1/21/F42.expansion.html ESS (rsna.org)].
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==IHC==
==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>
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.
*[[CD10]] +ve.
*h-caldesmin -ve.
*H-caldesmin -ve.
**[[Leiomyoma]]s +ve.
**[[Leiomyoma]]s +ve.
*PR +/-ve.
*PR +/-ve.
*ER +/-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==
==Molecular==

Latest revision as of 17:41, 20 March 2015

Endometrial stromal sarcoma
Diagnosis in short

Endometrial stromal sarcoma. H&E stain.
LM 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
LM DDx leiomyoma (cellular), endometrial stromal nodule, uterine leiomyosarcoma, uterine tumour resembling an ovarian sex cord stromal tumour
IHC CD10 +ve, H-caldesmin -ve, PR +/-ve, ER +/-ve
Molecular t(7;17)
Site uterus - see uterine tumours
Prevalence rare
Prognosis good
Clin. DDx other uterine tumours

Endometrial stromal sarcoma, abbreviated ESS, is a rare malignant mesenchymal tumour of the uterus.

It is also known as low-grade endometrial stromal sarcoma, abbreviated LGESS.

General

  • Uncommon.[1]
  • Prognosis good[2] > 90% five year survival and > 85% ten year survival.[3]

Note:

  • Extrauterine endometrial stromal sarcoma is known as endometrioid stromal sarcoma.[4]

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.[2]

DDx:

Images

  • ESS - low mag. (WC/Nephron)

  • ESS - low mag. (WC/Nephron)

  • ESS - intermed. mag. (WC/Nephron)

  • ESS - high mag. (WC/Nephron)

  • ESS - very high mag. (WC/Nephron)

  • ESS. (WC)

  • Endometrial stromal sarcoma - low grade. (WC)

www:

IHC

Features:[6]

Molecular

May be associated a recurrent translocation:[8]

  • t(7;17)(p15;q21).
    • JAZF1 - chromosome 7.[9]
    • SUZ12 - chromosome 17.[10]

See also

References

  1. ↑ Chew, I.; Oliva, E. (Mar 2010). "Endometrial stromal sarcomas: a review of potential prognostic factors.". Adv Anat Pathol 17 (2): 113-21. doi:10.1097/PAP.0b013e3181cfb7c2. PMID 20179433.
  2. ↑ 2.0 2.1 2.2 Feng, W.; Malpica, A.; Skaland, I.; Gudlaugsson, E.; Robboy, SJ.; Dalen, I.; Hua, K.; Zhou, X. et al. (2013). "Can proliferation biomarkers reliably predict recurrence in world health organization 2003 defined endometrial stromal sarcoma, low grade?". PLoS One 8 (10): e75899. doi:10.1371/journal.pone.0075899. PMID 24146786.
  3. ↑ Yoon, A.; Park, JY.; Park, JY.; Lee, YY.; Kim, TJ.; Choi, CH.; Bae, DS.; Kim, BG. et al. (Oct 2013). "Prognostic factors and outcomes in endometrial stromal sarcoma with the 2009 FIGO staging system: A multicenter review of 114 cases.". Gynecol Oncol. doi:10.1016/j.ygyno.2013.10.029. PMID 24184602.
  4. ↑ Masand, RP.; Euscher, ED.; Deavers, MT.; Malpica, A. (Nov 2013). "Endometrioid stromal sarcoma: a clinicopathologic study of 63 cases.". Am J Surg Pathol 37 (11): 1635-47. doi:10.1097/PAS.0000000000000083. PMID 24121169.
  5. ↑ Baker, P.; Oliva, E. (Mar 2007). "Endometrial stromal tumours of the uterus: a practical approach using conventional morphology and ancillary techniques.". J Clin Pathol 60 (3): 235-43. doi:10.1136/jcp.2005.031203. PMID 17347285. http://jcp.bmj.com/content/60/3/235.full.
  6. ↑ Zhu, XQ.; Shi, YF.; Cheng, XD.; Zhao, CL.; Wu, YZ. (Jan 2004). "Immunohistochemical markers in differential diagnosis of endometrial stromal sarcoma and cellular leiomyoma.". Gynecol Oncol 92 (1): 71-9. PMID 14751141.
  7. ↑ Park, JY.; Kim, KR.; Nam, JH. (Jan 2013). "Immunohistochemical analysis for therapeutic targets and prognostic markers in low-grade endometrial stromal sarcoma.". Int J Gynecol Cancer 23 (1): 81-9. doi:10.1097/IGC.0b013e3182738361. PMID 23221606.
  8. ↑ Amant, F.; Moerman, P.; Cadron, I.; Hagemeijer, A.; Vergote, I.; Debiec-Rychter, M. (Mar 2003). "Endometrial stromal sarcoma with a sole t(X;17) chromosome change: report of a case and review of the literature.". Gynecol Oncol 88 (3): 459-62. PMID 12648605.
  9. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 606246
  10. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 606245
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