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Uterine Corpus High Grade Endometrial Stromal Sarcoma

MedGen UID:
412175
•Concept ID:
C2239246
•
Neoplastic Process
Synonyms: Endometrial stromal sarcoma, high grade; Endometrial Stromal Sarcoma, High Grade; Endometrioid stromal sarcoma, high grade; High grade endometrial stromal sarcoma; High Grade ESS; Uterine Corpus High Grade ESS; YWHAE-FAM22 Endometrial Stromal Sarcoma; YWHAE::FAM22 Endometrial Stromal Sarcoma
SNOMED CT: Endometrial stromal sarcoma, high grade (70555003); High grade endometrial stromal sarcoma (699358009); Endometrioid stromal sarcoma, high grade (70555003)

Definition

A rare, high grade sarcoma that arises from the endometrial stroma. It is characterized by round cell morphology. It was previously also known as undifferentiated uterine sarcoma. In 2014, high grade endometrial stromal sarcoma was reclassified and is currently considered a distinct and rare neoplasm. It appears to have a prognosis that falls between low grade endometrial stromal sarcoma and undifferentiated sarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUterine Corpus High Grade Endometrial Stromal Sarcoma

Recent clinical studies

Etiology

High-grade transformation of low-grade endometrial stromal sarcomas lacking YWHAE and BCOR genetic abnormalities.
Zou Y, Turashvili G, Soslow RA, Park KJ, Croce S, McCluggage WG, Stewart CJR, Oda Y, Oliva E, Young RH, Da Cruz Paula A, Dessources K, Ashley CW, Hensley ML, Yip S, Weigelt B, Benayed R, Antonescu CR, Lee CH, Chiang S
Mod Pathol 2020 Sep;33(9):1861-1870. Epub 2020 Apr 21 doi: 10.1038/s41379-020-0535-y. PMID: 32317704Free PMC Article
Recent Developments in Surgical Pathology of the Uterine Corpus.
Hanley KZ, Birdsong GG, Mosunjac MB
Arch Pathol Lab Med 2017 Apr;141(4):528-541. Epub 2017 Feb 28 doi: 10.5858/arpa.2016-0284-SA. PMID: 28353387
Incidence and survival trends of uncommon corpus uteri malignancies in the Netherlands, 1989-2008.
Boll D, Verhoeven RH, van der Aa MA, Pauwels P, Karim-Kos HE, Coebergh JW, van Doorn HC
Int J Gynecol Cancer 2012 May;22(4):599-606. doi: 10.1097/IGC.0b013e318244cedc. PMID: 22398706
A multi-institutional review of outcomes of endometrial stromal sarcoma.
Leath CA 3rd, Huh WK, Hyde J Jr, Cohn DE, Resnick KE, Taylor NP, Powell MA, Mutch DG, Bradley WH, Geller MA, Argenta PA, Gold MA
Gynecol Oncol 2007 Jun;105(3):630-4. Epub 2007 Feb 23 doi: 10.1016/j.ygyno.2007.01.031. PMID: 17320937
Different types and different prognosis-study of 310 uterine sarcomas.
Nickie-Psikuta M, Gawrychowski K
Eur J Gynaecol Oncol 1993;14 Suppl:105-13. PMID: 8200360

Diagnosis

Pan-TRK immunohistochemistry in gynaecological mesenchymal tumours: diagnostic implications and pitfalls.
Moura MS, Costa J, Velasco V, Kommoss F, Oliva E, Le Loarer F, McCluggage WG, Razack R, Treilleux I, Mills A, Longacre T, Devouassoux-Shisheboran M, Hostein I, Azmani R, Blanchard L, Hartog C, Soubeyran I, Khalifa E, Croce S
Histopathology 2024 Feb;84(3):451-462. Epub 2023 Nov 21 doi: 10.1111/his.15082. PMID: 37988282
Description of a Novel ERBB4 -rearranged Uterine Sarcoma.
Lim SH, Mansor SB, Kathirvel R, Kuick CH, Lim-Tan SK, McCluggage WG
Int J Gynecol Pathol 2022 Sep 1;41(5):508-513. Epub 2021 Sep 27 doi: 10.1097/PGP.0000000000000826. PMID: 34570017
High-grade transformation of low-grade endometrial stromal sarcomas lacking YWHAE and BCOR genetic abnormalities.
Zou Y, Turashvili G, Soslow RA, Park KJ, Croce S, McCluggage WG, Stewart CJR, Oda Y, Oliva E, Young RH, Da Cruz Paula A, Dessources K, Ashley CW, Hensley ML, Yip S, Weigelt B, Benayed R, Antonescu CR, Lee CH, Chiang S
Mod Pathol 2020 Sep;33(9):1861-1870. Epub 2020 Apr 21 doi: 10.1038/s41379-020-0535-y. PMID: 32317704Free PMC Article
Recent Developments in Surgical Pathology of the Uterine Corpus.
Hanley KZ, Birdsong GG, Mosunjac MB
Arch Pathol Lab Med 2017 Apr;141(4):528-541. Epub 2017 Feb 28 doi: 10.5858/arpa.2016-0284-SA. PMID: 28353387
Endometrial stromal sarcoma of twenty cases.
Huang KT, Chen CA, Tseng GC, Chen TM, Cheng WF, Hsieh CY
Acta Obstet Gynecol Scand 1996 Jul;75(6):551-5. doi: 10.3109/00016349609054670. PMID: 8693932

Therapy

Endometrial stromal sarcoma of twenty cases.
Huang KT, Chen CA, Tseng GC, Chen TM, Cheng WF, Hsieh CY
Acta Obstet Gynecol Scand 1996 Jul;75(6):551-5. doi: 10.3109/00016349609054670. PMID: 8693932
Different types and different prognosis-study of 310 uterine sarcomas.
Nickie-Psikuta M, Gawrychowski K
Eur J Gynaecol Oncol 1993;14 Suppl:105-13. PMID: 8200360

Prognosis

Aggressive High-grade Uterine Sarcoma Harboring MEIS1-NCOA2 Fusion and Amplification of Multiple 12q13-15 Genes: A Case Report With Morphologic, Immunohistochemical, and Molecular Analysis.
Niu S, Rivera-Colon G, Lucas E
Int J Gynecol Pathol 2023 Sep 1;42(5):460-465. Epub 2023 Jan 25 doi: 10.1097/PGP.0000000000000937. PMID: 36811835
High-grade transformation of low-grade endometrial stromal sarcomas lacking YWHAE and BCOR genetic abnormalities.
Zou Y, Turashvili G, Soslow RA, Park KJ, Croce S, McCluggage WG, Stewart CJR, Oda Y, Oliva E, Young RH, Da Cruz Paula A, Dessources K, Ashley CW, Hensley ML, Yip S, Weigelt B, Benayed R, Antonescu CR, Lee CH, Chiang S
Mod Pathol 2020 Sep;33(9):1861-1870. Epub 2020 Apr 21 doi: 10.1038/s41379-020-0535-y. PMID: 32317704Free PMC Article
Recent Developments in Surgical Pathology of the Uterine Corpus.
Hanley KZ, Birdsong GG, Mosunjac MB
Arch Pathol Lab Med 2017 Apr;141(4):528-541. Epub 2017 Feb 28 doi: 10.5858/arpa.2016-0284-SA. PMID: 28353387
Mullerian adenosarcoma of the female genital tract.
McCluggage WG
Adv Anat Pathol 2010 Mar;17(2):122-9. doi: 10.1097/PAP.0b013e3181cfe732. PMID: 20179434
Endometrial stromal sarcoma of twenty cases.
Huang KT, Chen CA, Tseng GC, Chen TM, Cheng WF, Hsieh CY
Acta Obstet Gynecol Scand 1996 Jul;75(6):551-5. doi: 10.3109/00016349609054670. PMID: 8693932

Clinical prediction guides

Mixed Endometrioid Adenocarcinoma and Müllerian Adenosarcoma of the Uterus and Ovary: Clinicopathologic Characterization With Emphasis on its Distinction From Carcinosarcoma.
El Hallani S, Arora R, Lin DI, Måsbäc A, Mateoiu C, McCluggage WG, Nucci MR, Otis CN, Parkash V, Parra-Herran C, Longacre TA
Am J Surg Pathol 2021 Mar 1;45(3):374-383. doi: 10.1097/PAS.0000000000001643. PMID: 33565764
Incidence and survival trends of uncommon corpus uteri malignancies in the Netherlands, 1989-2008.
Boll D, Verhoeven RH, van der Aa MA, Pauwels P, Karim-Kos HE, Coebergh JW, van Doorn HC
Int J Gynecol Cancer 2012 May;22(4):599-606. doi: 10.1097/IGC.0b013e318244cedc. PMID: 22398706
Endometrial stromal sarcoma: clinicopathological and immunophenotype study of 18 cases.
Vera AA, Guadarrama MB
Ann Diagn Pathol 2011 Oct;15(5):312-7. Epub 2011 Jun 8 doi: 10.1016/j.anndiagpath.2011.01.008. PMID: 21652246
Fallopian tube cytology: a histocorrelative study of 150 washings.
Mulvany NJ, Arnstein M, Ostör AG
Diagn Cytopathol 1997 Jun;16(6):483-8. doi: 10.1002/(sici)1097-0339(199706)16:6<483::aid-dc3>3.0.co;2-b. PMID: 9181312

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