Endometrial stromal sarcoma

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

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
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

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]

  • CD10 +ve.
  • H-caldesmin -ve.
  • PR +/-ve.
  • ER +/-ve.
  • CD117 +ve (~80%).[7]

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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