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Ovarian Undifferentiated Sarcoma

MedGen UID:
384493
•Concept ID:
C2188139
•
Neoplastic Process
Synonym: Undifferentiated Ovarian Sarcoma

Definition

An aggressive high grade sarcoma that arises from the ovary. It is characterized by the presence of marked pleomorphism and nuclear atypia in the neoplastic mesenchymal cells. The prognosis is poor. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Undifferentiated Sarcoma

Professional guidelines

PubMed

Endometrial stromal tumors of the uterus: Epidemiology, pathological and biological features, treatment options and clinical outcomes.
Gadducci A, Multinu F, De Vitis LA, Cosio S, Carinelli S, Aletti GD
Gynecol Oncol 2023 Apr;171:95-105. Epub 2023 Feb 24 doi: 10.1016/j.ygyno.2023.02.009. PMID: 36842409
The value of SOX2 in the differential diagnosis of SMARCA4 (BRG1)-deficient uterine neoplasms.
Zheng L, Zhang L, Wang XI, Katz G, Tandon N, Zhao B, Lucci J, Ding J, Zhang S
Hum Pathol 2022 Jun;124:45-55. Epub 2022 Mar 22 doi: 10.1016/j.humpath.2022.03.009. PMID: 35331811
Treatment options in stage I endometrial stromal sarcoma: a retrospective analysis of 53 cases.
Li N, Wu LY, Zhang HT, An JS, Li XG, Ma SK
Gynecol Oncol 2008 Feb;108(2):306-11. Epub 2007 Dec 3 doi: 10.1016/j.ygyno.2007.10.023. PMID: 18061249

Recent clinical studies

Etiology

Gynecological lesions in hereditary cancer predisposition syndromes.
Dundr P, Cibula D, Foretová L, Macek M Jr, Kopečková K, Petruželka L, Němejcová K, Bártů M, Hojný J, Hájková N, Jakša R, Janega P, Stružinská I
Cesk Patol 2021 Summer;57(2):96-104. PMID: 34275319
Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020.
Landry JP, Schertz KL, Chiang YJ, Bhalla AD, Yi M, Keung EZ, Scally CP, Feig BW, Hunt KK, Roland CL, Guadagnolo A, Bishop AJ, Lazar AJ, Slopis JM, McCutcheon IE, Torres KE
JAMA Netw Open 2021 Mar 1;4(3):e210945. doi: 10.1001/jamanetworkopen.2021.0945. PMID: 33734413Free PMC Article
Undifferentiated and dedifferentiated neoplasms of the female genital tract.
Bennett JA, Oliva E
Semin Diagn Pathol 2021 Nov;38(6):137-151. Epub 2020 Nov 27 doi: 10.1053/j.semdp.2020.11.002. PMID: 33323288
Beyond Smooth Muscle-Other Mesenchymal Neoplasms of the Uterus.
Dickson BC
Surg Pathol Clin 2019 Mar;12(1):107-137. Epub 2018 Dec 24 doi: 10.1016/j.path.2018.10.005. PMID: 30709439
Cellular mesenchymal tumors of the uterus: a review emphasizing recent observations.
Oliva E
Int J Gynecol Pathol 2014 Jul;33(4):374-84. doi: 10.1097/PGP.0000000000000141. PMID: 24901397

Diagnosis

Special features of sarcomas developed in patients with Lynch syndrome: A systematic review.
Poumeaud F, Valentin T, Vande Perre P, Jaffrelot M, Bonnet D, Chibon F, Chevreau C, Selves J, Guimbaud R, Fares N
Crit Rev Oncol Hematol 2023 Aug;188:104055. Epub 2023 Jun 8 doi: 10.1016/j.critrevonc.2023.104055. PMID: 37301271
Endometrial stromal tumors of the uterus: Epidemiology, pathological and biological features, treatment options and clinical outcomes.
Gadducci A, Multinu F, De Vitis LA, Cosio S, Carinelli S, Aletti GD
Gynecol Oncol 2023 Apr;171:95-105. Epub 2023 Feb 24 doi: 10.1016/j.ygyno.2023.02.009. PMID: 36842409
Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020.
Landry JP, Schertz KL, Chiang YJ, Bhalla AD, Yi M, Keung EZ, Scally CP, Feig BW, Hunt KK, Roland CL, Guadagnolo A, Bishop AJ, Lazar AJ, Slopis JM, McCutcheon IE, Torres KE
JAMA Netw Open 2021 Mar 1;4(3):e210945. doi: 10.1001/jamanetworkopen.2021.0945. PMID: 33734413Free PMC Article
Undifferentiated and dedifferentiated neoplasms of the female genital tract.
Bennett JA, Oliva E
Semin Diagn Pathol 2021 Nov;38(6):137-151. Epub 2020 Nov 27 doi: 10.1053/j.semdp.2020.11.002. PMID: 33323288
Practical issues related to uterine pathology: endometrial stromal tumors.
Nucci MR
Mod Pathol 2016 Jan;29 Suppl 1:S92-103. doi: 10.1038/modpathol.2015.140. PMID: 26715176

Therapy

HER2 testing of gynecologic carcinosarcomas: tumor stratification for potential targeted therapy.
Rottmann D, Snir OL, Wu X, Wong S, Hui P, Santin AD, Buza N
Mod Pathol 2020 Jan;33(1):118-127. Epub 2019 Sep 2 doi: 10.1038/s41379-019-0358-x. PMID: 31477811
A rare case of ovarian carcinosarcoma with squamous cell carcinoma.
Daimon A, Fujiwara S, Tanaka Y, Tanaka T, Ohmichi M
J Ovarian Res 2019 Apr 4;12(1):32. doi: 10.1186/s13048-019-0507-3. PMID: 30947745Free PMC Article
Treatment options in stage I endometrial stromal sarcoma: a retrospective analysis of 53 cases.
Li N, Wu LY, Zhang HT, An JS, Li XG, Ma SK
Gynecol Oncol 2008 Feb;108(2):306-11. Epub 2007 Dec 3 doi: 10.1016/j.ygyno.2007.10.023. PMID: 18061249
Ovarian sarcoma with pathologic features of clear cell sarcoma of the kidney.
Finn LS, Patterson K
Pediatr Dev Pathol 2000 Sep-Oct;3(5):487-91. doi: 10.1007/s100240010095. PMID: 10890934
Ovarian mucinous cystadenocarcinoma with sarcoma-appearing mural nodule of anaplastic carcinoma.
Tsuruchi N, Kaku T, Kinoshita H, Amada S, Saito T, Kamura T, Tsukamoto N, Nakano H
Gynecol Oncol 1993 Aug;50(2):259-63. doi: 10.1006/gyno.1993.1204. PMID: 8375741

Prognosis

Endometrial stromal tumors of the uterus: Epidemiology, pathological and biological features, treatment options and clinical outcomes.
Gadducci A, Multinu F, De Vitis LA, Cosio S, Carinelli S, Aletti GD
Gynecol Oncol 2023 Apr;171:95-105. Epub 2023 Feb 24 doi: 10.1016/j.ygyno.2023.02.009. PMID: 36842409
The value of SOX2 in the differential diagnosis of SMARCA4 (BRG1)-deficient uterine neoplasms.
Zheng L, Zhang L, Wang XI, Katz G, Tandon N, Zhao B, Lucci J, Ding J, Zhang S
Hum Pathol 2022 Jun;124:45-55. Epub 2022 Mar 22 doi: 10.1016/j.humpath.2022.03.009. PMID: 35331811
Beyond Smooth Muscle-Other Mesenchymal Neoplasms of the Uterus.
Dickson BC
Surg Pathol Clin 2019 Mar;12(1):107-137. Epub 2018 Dec 24 doi: 10.1016/j.path.2018.10.005. PMID: 30709439
SMARCA4-deficient undifferentiated uterine sarcoma (malignant rhabdoid tumor of the uterus): a clinicopathologic entity distinct from undifferentiated carcinoma.
Kolin DL, Dong F, Baltay M, Lindeman N, MacConaill L, Nucci MR, Crum CP, Howitt BE
Mod Pathol 2018 Sep;31(9):1442-1456. Epub 2018 Apr 26 doi: 10.1038/s41379-018-0049-z. PMID: 29700418
Endometrial and ovarian carcinomas with undifferentiated components: clinically aggressive and frequently underrecognized neoplasms.
Tafe LJ, Garg K, Chew I, Tornos C, Soslow RA
Mod Pathol 2010 Jun;23(6):781-9. Epub 2010 Mar 19 doi: 10.1038/modpathol.2010.41. PMID: 20305618

Clinical prediction guides

Mesonephric-like adenocarcinoma of the female genital tract: novel observations and detailed molecular characterisation of mixed tumours and mesonephric-like carcinosarcomas.
Mirkovic J, Olkhov-Mitsel E, Amemiya Y, Al-Hussaini M, Nofech-Mozes S, Djordjevic B, Kupets R, Seth A, McCluggage WG
Histopathology 2023 Jun;82(7):978-990. Epub 2023 Mar 31 doi: 10.1111/his.14892. PMID: 36860193
Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020.
Landry JP, Schertz KL, Chiang YJ, Bhalla AD, Yi M, Keung EZ, Scally CP, Feig BW, Hunt KK, Roland CL, Guadagnolo A, Bishop AJ, Lazar AJ, Slopis JM, McCutcheon IE, Torres KE
JAMA Netw Open 2021 Mar 1;4(3):e210945. doi: 10.1001/jamanetworkopen.2021.0945. PMID: 33734413Free PMC Article
Loss of ARID1B and SMARCB1 expression are specific for the diagnosis of dedifferentiated/undifferentiated carcinoma in tumours of the upper gynaecological tract and cervix.
Kang EY, Tessier-Cloutier B, Duggan MA, Stewart CJR, Lee CH, Köbel M
Histopathology 2021 Aug;79(2):160-167. Epub 2021 Jun 2 doi: 10.1111/his.14333. PMID: 33432679
Cellular mesenchymal tumors of the uterus: a review emphasizing recent observations.
Oliva E
Int J Gynecol Pathol 2014 Jul;33(4):374-84. doi: 10.1097/PGP.0000000000000141. PMID: 24901397
Endometrial and ovarian carcinomas with undifferentiated components: clinically aggressive and frequently underrecognized neoplasms.
Tafe LJ, Garg K, Chew I, Tornos C, Soslow RA
Mod Pathol 2010 Jun;23(6):781-9. Epub 2010 Mar 19 doi: 10.1038/modpathol.2010.41. PMID: 20305618

Recent systematic reviews

Special features of sarcomas developed in patients with Lynch syndrome: A systematic review.
Poumeaud F, Valentin T, Vande Perre P, Jaffrelot M, Bonnet D, Chibon F, Chevreau C, Selves J, Guimbaud R, Fares N
Crit Rev Oncol Hematol 2023 Aug;188:104055. Epub 2023 Jun 8 doi: 10.1016/j.critrevonc.2023.104055. PMID: 37301271

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