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Uterine Corpus Low Grade Endometrial Stromal Sarcoma

MedGen UID:
275557
•Concept ID:
C1519857
•
Neoplastic Process
Synonyms: JAZF1-SUZ12 Endometrial Stromal Sarcoma; JAZF1::SUZ12 Endometrial Stromal Sarcoma; Uterine Corpus Low Grade ESS

Definition

A rare, indolent, invasive mesenchymal tumor that arises from the endometrial stroma. It is characterized by the presence of a plexiform vasculature, infrequent mitoses, and insignificant cytologic atypia. Late recurrences may occur. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Extrauterine Spread, Adjuvant Treatment, and Prognosis in Noninvasive Uterine Papillary Serous Carcinoma of the Endometrium: A Retrospective Multicenter Study.
Boyraz G, Salman MC, Basaran D, Ozgul N, Turan T, Turkmen O, Kimyon GC, Ozkan NT, Cagan M, Gungor T, Meydanli MM, Gungorduk K, Gulseren V, Sanci M, Yuce K
Int J Gynecol Cancer 2017 Jan;27(1):102-108. doi: 10.1097/IGC.0000000000000838. PMID: 27668395
Uterine neoplasms, version 1.2014.
Koh WJ, Greer BE, Abu-Rustum NR, Apte SM, Campos SM, Chan J, Cho KR, Cohn D, Crispens MA, Dupont N, Eifel PJ, Fader AN, Fisher CM, Gaffney DK, George S, Han E, Huh WK, Lurain JR 3rd, Martin L, Mutch D, Remmenga SW, Reynolds RK, Small W Jr, Teng N, Tillmanns T, Valea FA, McMillian N, Hughes M
J Natl Compr Canc Netw 2014 Feb;12(2):248-80. doi: 10.6004/jnccn.2014.0025. PMID: 24586086
Results of postoperative radiotherapy in the treatment of sarcoma of the corpus uteri.
Knocke TH, Kucera H, Dörfler D, Pokrajac B, Pötter R
Cancer 1998 Nov 1;83(9):1972-9. doi: 10.1002/(sici)1097-0142(19981101)83:9<1972::aid-cncr13>3.0.co;2-m. PMID: 9806656

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
Uterine neoplasms, version 1.2014.
Koh WJ, Greer BE, Abu-Rustum NR, Apte SM, Campos SM, Chan J, Cho KR, Cohn D, Crispens MA, Dupont N, Eifel PJ, Fader AN, Fisher CM, Gaffney DK, George S, Han E, Huh WK, Lurain JR 3rd, Martin L, Mutch D, Remmenga SW, Reynolds RK, Small W Jr, Teng N, Tillmanns T, Valea FA, McMillian N, Hughes M
J Natl Compr Canc Netw 2014 Feb;12(2):248-80. doi: 10.6004/jnccn.2014.0025. PMID: 24586086
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Sarcomas of the female genital tract and pelvic soft tissues. A cluster of 14 cases in one year.
Gleeson N, Gaffney EF, Gonsalves R, Quigley J, Bonnar J
Ir Med J 1993 Nov-Dec;86(6):196-8. PMID: 8106227

Diagnosis

Low-grade endometrial stromal sarcoma-like tumors in male with JAZF1 gene fusions.
Dermawan JK, Zhang L, Singer S, Chi P, Antonescu CR
Genes Chromosomes Cancer 2022 Feb;61(2):63-70. Epub 2021 Oct 23 doi: 10.1002/gcc.23003. PMID: 34651371Free PMC Article
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
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Pure mesenchymal neoplasms of the uterine corpus: selected problems.
Kempson RL, Hendrickson MR
Semin Diagn Pathol 1988 May;5(2):172-98. PMID: 3041510

Therapy

Tamoxifen related Uterine Tumor Resembling Ovarian Sex Cord Tumor (UTROSCT): A case report and literature review of this possible association.
Segala D, Gobbo S, Pesci A, Martignoni G, Santoro A, Angelico G, Arciuolo D, Spadola S, Valente M, Scambia G, Zannoni GF
Pathol Res Pract 2019 May;215(5):1089-1092. Epub 2019 Jan 28 doi: 10.1016/j.prp.2019.01.039. PMID: 30799070
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

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
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
FIGO staging of endometrial adenocarcinoma: a critical review and proposal.
Zaino RJ
Int J Gynecol Pathol 2009 Jan;28(1):1-9. doi: 10.1097/PGP.0b013e3181846c6d. PMID: 19047915
Pure mesenchymal neoplasms of the uterine corpus: selected problems.
Kempson RL, Hendrickson MR
Semin Diagn Pathol 1988 May;5(2):172-98. PMID: 3041510

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
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
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Uterine adenomyoma: a clinicopathologic review of 26 cases and a review of the literature.
Tahlan A, Nanda A, Mohan H
Int J Gynecol Pathol 2006 Oct;25(4):361-5. doi: 10.1097/01.pgp.0000209570.08716.b3. PMID: 16990713
Pure mesenchymal neoplasms of the uterine corpus: selected problems.
Kempson RL, Hendrickson MR
Semin Diagn Pathol 1988 May;5(2):172-98. PMID: 3041510

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