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
Clin. DDx other uterine tumours

It is also known as low-grade endometrial stromal sarcoma

General

  • Uncommon.[1]
  • Prognosis good.[2]

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

www:

IHC

Features:[4]

  • CD10 +ve.
  • H-caldesmin -ve.
  • PR +/-ve.
  • ER +/-ve.

Molecular

May be associated a recurrent translocation:[5]

  • t(7;17)(p15;q21).
    • JAZF1 - chromosome 7.[6]
    • SUZ12 - chromosome 17.[7]

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 Cite error: Invalid <ref> tag; no text was provided for refs named pmid24146786
  3. 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.
  4. 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.
  5. 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.
  6. Online 'Mendelian Inheritance in Man' (OMIM) 606246
  7. Online 'Mendelian Inheritance in Man' (OMIM) 606245