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Uterine Corpus Undifferentiated Sarcoma

MedGen UID:
412175
•Concept ID:
C2239246
•
Neoplastic Process
Synonyms: Endometrial stromal sarcoma, high grade; Endometrioid stromal sarcoma, high grade; High grade endometrial stromal sarcoma; Undifferentiated Uterine Sarcoma; Uterine Corpus Undifferentiated Endometrial 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 or myometrium without a specific type of differentiation. It was previously also known as high grade endometrial stromal sarcoma. In 2014, high grade endometrial stromal sarcoma was reclassified and is currently considered a distinct and rare neoplasm. Undifferentiated uterine sarcoma has a worse prognosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUterine Corpus Undifferentiated Sarcoma

Professional guidelines

PubMed

Uterine Neoplasms, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology.
Abu-Rustum N, Yashar C, Arend R, Barber E, Bradley K, Brooks R, Campos SM, Chino J, Chon HS, Chu C, Crispens MA, Damast S, Fisher CM, Frederick P, Gaffney DK, Giuntoli R, Han E, Holmes J, Howitt BE, Lea J, Mariani A, Mutch D, Nagel C, Nekhlyudov L, Podoll M, Salani R, Schorge J, Siedel J, Sisodia R, Soliman P, Ueda S, Urban R, Wethington SL, Wyse E, Zanotti K, McMillian NR, Aggarwal S
J Natl Compr Canc Netw 2023 Feb;21(2):181-209. doi: 10.6004/jnccn.2023.0006. PMID: 36791750

Recent clinical studies

Etiology

Uterine Neoplasms, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology.
Abu-Rustum N, Yashar C, Arend R, Barber E, Bradley K, Brooks R, Campos SM, Chino J, Chon HS, Chu C, Crispens MA, Damast S, Fisher CM, Frederick P, Gaffney DK, Giuntoli R, Han E, Holmes J, Howitt BE, Lea J, Mariani A, Mutch D, Nagel C, Nekhlyudov L, Podoll M, Salani R, Schorge J, Siedel J, Sisodia R, Soliman P, Ueda S, Urban R, Wethington SL, Wyse E, Zanotti K, McMillian NR, Aggarwal S
J Natl Compr Canc Netw 2023 Feb;21(2):181-209. doi: 10.6004/jnccn.2023.0006. PMID: 36791750
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
SATB2 Expression Is Sensitive but Not Specific for Osteosarcomatous Components of Gynecologic Tract Carcinosarcomas: A Clinicopathologic Study of 60 Cases.
Sangoi AR, Kshirsagar M, Horvai AE, Roma AA
Int J Gynecol Pathol 2017 Mar;36(2):140-145. doi: 10.1097/PGP.0000000000000301. PMID: 27294605
Cytoenzymology of benign and malignant tumours of the corpus uteri. I. Respiratory enzymes.
El-Fiky SM, Taha YM
Acta Histochem 1976;56(1):1-17. doi: 10.1016/S0065-1281(76)80022-0. PMID: 184637

Diagnosis

Uterine Neoplasms, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology.
Abu-Rustum N, Yashar C, Arend R, Barber E, Bradley K, Brooks R, Campos SM, Chino J, Chon HS, Chu C, Crispens MA, Damast S, Fisher CM, Frederick P, Gaffney DK, Giuntoli R, Han E, Holmes J, Howitt BE, Lea J, Mariani A, Mutch D, Nagel C, Nekhlyudov L, Podoll M, Salani R, Schorge J, Siedel J, Sisodia R, Soliman P, Ueda S, Urban R, Wethington SL, Wyse E, Zanotti K, McMillian NR, Aggarwal S
J Natl Compr Canc Netw 2023 Feb;21(2):181-209. doi: 10.6004/jnccn.2023.0006. PMID: 36791750
ZEB1 expression is frequently detected in undifferentiated and de-differentiated carcinomas, but is not specific among endometrial carcinomas.
Kihara A, Amano Y, Fukushima N, Fujiwara H, Niki T
Histopathology 2022 Jul;81(1):108-118. Epub 2022 May 18 doi: 10.1111/his.14671. PMID: 35478361
Dedifferentiated Mesonephric-like Adenocarcinoma of the Uterine Corpus.
Choi S, Na K, Kim SW, Kim HS
Anticancer Res 2021 May;41(5):2719-2726. doi: 10.21873/anticanres.15053. PMID: 33952503
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

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
ZEB1 expression is frequently detected in undifferentiated and de-differentiated carcinomas, but is not specific among endometrial carcinomas.
Kihara A, Amano Y, Fukushima N, Fujiwara H, Niki T
Histopathology 2022 Jul;81(1):108-118. Epub 2022 May 18 doi: 10.1111/his.14671. PMID: 35478361
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
Uterine adenosarcoma with sarcomatous overgrowth: a case report with cytology of overgrown poorly differentiated sarcoma and immunohistochemical identification of epithelial microinvasion.
Nagai Y, Kishimoto T, Kato K, Ozaki D, Kondo F, Kobayashi A, Shimizu H, Ishikura H
Int J Gynecol Cancer 2002 Sep-Oct;12(5):501-5. doi: 10.1046/j.1525-1438.2002.01129.x. PMID: 12366670

Clinical prediction guides

ZEB1 expression is frequently detected in undifferentiated and de-differentiated carcinomas, but is not specific among endometrial carcinomas.
Kihara A, Amano Y, Fukushima N, Fujiwara H, Niki T
Histopathology 2022 Jul;81(1):108-118. Epub 2022 May 18 doi: 10.1111/his.14671. PMID: 35478361
Dedifferentiated Mesonephric-like Adenocarcinoma of the Uterine Corpus.
Choi S, Na K, Kim SW, Kim HS
Anticancer Res 2021 May;41(5):2719-2726. doi: 10.21873/anticanres.15053. PMID: 33952503
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
Cytoenzymology of benign and malignant tumours of the corpus uteri. I. Respiratory enzymes.
El-Fiky SM, Taha YM
Acta Histochem 1976;56(1):1-17. doi: 10.1016/S0065-1281(76)80022-0. PMID: 184637

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