Endometrial stromal sarcoma

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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

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