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Ovarian carcinosarcoma

MedGen UID:
140705
•Concept ID:
C0392998
•
Neoplastic Process
Synonyms: Carcinosarcoma of ovary; Carcinosarcoma of Ovary; carcinosarcoma of ovary; Carcinosarcoma of the Ovary; carcinosarcoma of the ovary; Malignant Mixed Mesodermal Mullerian Neoplasm of Ovary; malignant mixed mesodermal Mullerian neoplasm of ovary; Malignant Mixed Mesodermal Mullerian Neoplasm of the Ovary; malignant mixed mesodermal Mullerian neoplasm of the ovary; Malignant Mixed Mesodermal Mullerian Tumor of Ovary; malignant mixed mesodermal Mullerian tumor of ovary; Malignant Mixed Mesodermal Mullerian Tumor of the Ovary; malignant mixed mesodermal Mullerian tumor of the ovary; malignant mixed mesodermal Mullerian tumour of ovary; malignant mixed mesodermal Mullerian tumour of the ovary; Malignant Mixed Mesodermal Müllerian Neoplasm of Ovary; malignant mixed mesodermal Müllerian neoplasm of ovary; Malignant Mixed Mesodermal Müllerian Neoplasm of the Ovary; malignant mixed mesodermal Müllerian neoplasm of the ovary; Malignant Mixed Mesodermal Müllerian Tumor of Ovary; malignant mixed mesodermal Müllerian tumor of ovary; Malignant Mixed Mesodermal Müllerian Tumor of the Ovary; malignant mixed mesodermal Müllerian tumor of the ovary; malignant mixed mesodermal Müllerian tumour of ovary; malignant mixed mesodermal Müllerian tumour of the ovary; malignant mixed Müllerian tumor of the ovary; Malignant mixed Müllerian tumor of the ovary; malignant mixed Müllerian tumour of the ovary; MMMT of the ovary; Ovarian Carcinosarcoma; ovarian carcinosarcoma; ovarian carcinosarcoma/malignant mixed mesodermal tumor; ovarian carcinosarcoma/malignant mixed mesodermal tumour; Ovarian Malignant Mesodermal (Mullerian) Mixed Tumor; ovarian malignant mesodermal (Mullerian) mixed tumor; ovarian malignant mesodermal (mullerian) mixed tumor; ovarian malignant mesodermal (mullerian) mixed tumour; ovarian malignant mesodermal (Mullerian) mixed tumour; Ovarian Malignant Mesodermal (Müllerian) Mixed Tumor; ovarian malignant mesodermal (Müllerian) mixed tumor; ovarian malignant mesodermal (Müllerian) mixed tumour; Ovarian malignant mixed epithelial mesenchymal tumor; ovarian malignant mixed epithelial mesenchymal tumor; ovarian malignant mixed epithelial mesenchymal tumour; Ovarian Malignant Mixed Mesodermal (Mullerian) Tumor; ovarian malignant mixed mesodermal (Mullerian) tumor; ovarian malignant mixed mesodermal (Mullerian) tumour; Ovarian Malignant Mixed Mesodermal (Müllerian) Tumor; ovarian malignant mixed mesodermal (Müllerian) tumor; ovarian malignant mixed mesodermal (Müllerian) tumour; Ovarian Malignant Mixed Mesodermal Mullerian Neoplasm; ovarian malignant mixed mesodermal Mullerian neoplasm; Ovarian Malignant Mixed Mesodermal Mullerian Tumor; ovarian malignant mixed mesodermal Mullerian tumor; ovarian malignant mixed mesodermal Mullerian tumour; Ovarian Malignant Mixed Mesodermal Müllerian Neoplasm; ovarian malignant mixed mesodermal Müllerian neoplasm; Ovarian Malignant Mixed Mesodermal Müllerian Tumor; ovarian malignant mixed mesodermal Müllerian tumor; ovarian malignant mixed mesodermal Müllerian tumour; Ovarian Malignant Mixed Mullerian Neoplasm; ovarian malignant mixed Mullerian neoplasm; Ovarian Malignant Mixed Mullerian Tumor; ovarian malignant mixed Mullerian tumor; ovarian malignant mixed Mullerian tumour; Ovarian Malignant Mixed Müllerian Neoplasm; ovarian malignant mixed Müllerian neoplasm; Ovarian Malignant Mixed Müllerian Tumor; Ovarian malignant mixed Müllerian tumor; ovarian malignant mixed Müllerian tumor; ovarian malignant mixed Müllerian tumour; ovarian MMMT; ovary carcinosarcoma
SNOMED CT: Carcinosarcoma of ovary (702368000)
 
Monarch Initiative: MONDO:0003792
Orphanet: ORPHA213512

Definition

Malignant mixed Müllerian tumor of the ovary is a rare and very aggressive neoplasm presenting most commonly in postmenopausal women and is composed of adenocarcinomatous and sarcomatous elements and, depending on the types of these elements, can be classified as homologous or heterologous. It often has a poor prognosis. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian carcinosarcoma

Professional guidelines

PubMed

T-Cell Receptor Therapy in the Treatment of Ovarian Cancer: A Mini Review.
Wu JWY, Dand S, Doig L, Papenfuss AT, Scott CL, Ho G, Ooi JD
Front Immunol 2021;12:672502. Epub 2021 Apr 13 doi: 10.3389/fimmu.2021.672502. PMID: 33927729Free PMC Article
"Carcinosarcoma of the ovary, fallopian tube, and peritoneum: Prognostic factors and treatment modalities".
Rauh-Hain JA, Birrer M, Del Carmen MG
Gynecol Oncol 2016 Aug;142(2):248-54. Epub 2016 Jun 16 doi: 10.1016/j.ygyno.2016.06.003. PMID: 27321238
Current management of ovarian carcinosarcoma.
Mano MS, Rosa DD, Azambuja E, Ismael G, Braga S, D'Hondt V, Piccart M, Awada A
Int J Gynecol Cancer 2007 Mar-Apr;17(2):316-24. doi: 10.1111/j.1525-1438.2006.00760.x. PMID: 17362309

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Ovarian Cancer Including Fallopian Tube Cancer and Primary Peritoneal Cancer, 2024

Recent clinical studies

Etiology

Frontiers of Ovarian Carcinosarcoma.
Ismail A, Choi S, Boussios S
Curr Treat Options Oncol 2023 Dec;24(12):1667-1682. Epub 2023 Nov 8 doi: 10.1007/s11864-023-01138-4. PMID: 37938504Free PMC Article
Folate receptor alpha is widely expressed and a potential therapeutic target in uterine and ovarian carcinosarcoma.
Saito A, Nishikawa T, Yoshida H, Mizoguchi C, Kitadai R, Yamamoto K, Yazaki S, Kojima Y, Ishikawa M, Kato T, Yonemori K
Gynecol Oncol 2023 Sep;176:115-121. Epub 2023 Jul 26 doi: 10.1016/j.ygyno.2023.07.010. PMID: 37506533
Germline drivers of gynecologic carcinosarcomas.
Sia TY, Gordhandas SB, Birsoy O, Kemel Y, Maio A, Salo-Mullen E, Sheehan M, Hensley ML, Rubinstein M, Makker V, Grisham RN, O'Cearbhaill RE, Roche KL, Mueller JJ, Leitao MM Jr, Sonoda Y, Chi DS, Abu-Rustum NR, Berger MF, Ellenson LH, Latham A, Stadler Z, Offit K, Aghajanian C, Weigelt B, Mandelker D, Liu YL
Gynecol Oncol 2023 Jul;174:34-41. Epub 2023 May 5 doi: 10.1016/j.ygyno.2023.04.024. PMID: 37149903Free PMC Article
Detailed overview on rare malignant ovarian tumors.
Aust S, Eberst L, Tredan O, Rousset-Jablonski C, Treilleux I, Méeus P, Chopin N, Beurrier F, Charreton A, Véronique D, Hallouz A, Coulon A, Ricoeur A, Mastier C, Bouhamama A, Racadot S, Devouassoux-Shisheboran M, Haddad V, Ray-Coquard I
Bull Cancer 2020 Mar;107(3):385-390. Epub 2020 Feb 27 doi: 10.1016/j.bulcan.2020.01.011. PMID: 32115180
"Carcinosarcoma of the ovary, fallopian tube, and peritoneum: Prognostic factors and treatment modalities".
Rauh-Hain JA, Birrer M, Del Carmen MG
Gynecol Oncol 2016 Aug;142(2):248-54. Epub 2016 Jun 16 doi: 10.1016/j.ygyno.2016.06.003. PMID: 27321238

Diagnosis

Frontiers of Ovarian Carcinosarcoma.
Ismail A, Choi S, Boussios S
Curr Treat Options Oncol 2023 Dec;24(12):1667-1682. Epub 2023 Nov 8 doi: 10.1007/s11864-023-01138-4. PMID: 37938504Free PMC Article
Germline drivers of gynecologic carcinosarcomas.
Sia TY, Gordhandas SB, Birsoy O, Kemel Y, Maio A, Salo-Mullen E, Sheehan M, Hensley ML, Rubinstein M, Makker V, Grisham RN, O'Cearbhaill RE, Roche KL, Mueller JJ, Leitao MM Jr, Sonoda Y, Chi DS, Abu-Rustum NR, Berger MF, Ellenson LH, Latham A, Stadler Z, Offit K, Aghajanian C, Weigelt B, Mandelker D, Liu YL
Gynecol Oncol 2023 Jul;174:34-41. Epub 2023 May 5 doi: 10.1016/j.ygyno.2023.04.024. PMID: 37149903Free PMC Article
Ovarian carcinosarcoma: Current developments and future perspectives.
Boussios S, Karathanasi A, Zakynthinakis-Kyriakou N, Tsiouris AK, Chatziantoniou AA, Kanellos FS, Tatsi K
Crit Rev Oncol Hematol 2019 Feb;134:46-55. Epub 2018 Dec 24 doi: 10.1016/j.critrevonc.2018.12.006. PMID: 30771873
"Carcinosarcoma of the ovary, fallopian tube, and peritoneum: Prognostic factors and treatment modalities".
Rauh-Hain JA, Birrer M, Del Carmen MG
Gynecol Oncol 2016 Aug;142(2):248-54. Epub 2016 Jun 16 doi: 10.1016/j.ygyno.2016.06.003. PMID: 27321238
Current management of ovarian carcinosarcoma.
Mano MS, Rosa DD, Azambuja E, Ismael G, Braga S, D'Hondt V, Piccart M, Awada A
Int J Gynecol Cancer 2007 Mar-Apr;17(2):316-24. doi: 10.1111/j.1525-1438.2006.00760.x. PMID: 17362309

Therapy

Genomic and Molecular Characteristics of Ovarian Carcinosarcoma.
Ramphal K, Hadfield MJ, Bandera CM, Hart J, Dizon DS
Am J Clin Oncol 2023 Dec 1;46(12):572-576. Epub 2023 Oct 17 doi: 10.1097/COC.0000000000001056. PMID: 37986208
Relacorilant + Nab-Paclitaxel in Patients With Recurrent, Platinum-Resistant Ovarian Cancer: A Three-Arm, Randomized, Controlled, Open-Label Phase II Study.
Colombo N, Van Gorp T, Matulonis UA, Oaknin A, Grisham RN, Fleming GF, Olawaiye AB, Nguyen DD, Greenstein AE, Custodio JM, Pashova HI, Tudor IC, Lorusso D
J Clin Oncol 2023 Oct 20;41(30):4779-4789. Epub 2023 Jun 26 doi: 10.1200/JCO.22.02624. PMID: 37364223Free PMC Article
Randomized Phase III Trial of Paclitaxel and Carboplatin Versus Paclitaxel and Ifosfamide in Patients With Carcinosarcoma of the Uterus or Ovary: An NRG Oncology Trial.
Powell MA, Filiaci VL, Hensley ML, Huang HQ, Moore KN, Tewari KS, Copeland LJ, Secord AA, Mutch DG, Santin A, Warshal DP, Spirtos NM, DiSilvestro PA, Ioffe OB, Miller DS
J Clin Oncol 2022 Mar 20;40(9):968-977. Epub 2022 Jan 10 doi: 10.1200/JCO.21.02050. PMID: 35007153Free PMC Article
T-Cell Receptor Therapy in the Treatment of Ovarian Cancer: A Mini Review.
Wu JWY, Dand S, Doig L, Papenfuss AT, Scott CL, Ho G, Ooi JD
Front Immunol 2021;12:672502. Epub 2021 Apr 13 doi: 10.3389/fimmu.2021.672502. PMID: 33927729Free PMC Article
Current management of ovarian carcinosarcoma.
Mano MS, Rosa DD, Azambuja E, Ismael G, Braga S, D'Hondt V, Piccart M, Awada A
Int J Gynecol Cancer 2007 Mar-Apr;17(2):316-24. doi: 10.1111/j.1525-1438.2006.00760.x. PMID: 17362309

Prognosis

Genomic and Molecular Characteristics of Ovarian Carcinosarcoma.
Ramphal K, Hadfield MJ, Bandera CM, Hart J, Dizon DS
Am J Clin Oncol 2023 Dec 1;46(12):572-576. Epub 2023 Oct 17 doi: 10.1097/COC.0000000000001056. PMID: 37986208
Frontiers of Ovarian Carcinosarcoma.
Ismail A, Choi S, Boussios S
Curr Treat Options Oncol 2023 Dec;24(12):1667-1682. Epub 2023 Nov 8 doi: 10.1007/s11864-023-01138-4. PMID: 37938504Free PMC Article
Folate receptor alpha is widely expressed and a potential therapeutic target in uterine and ovarian carcinosarcoma.
Saito A, Nishikawa T, Yoshida H, Mizoguchi C, Kitadai R, Yamamoto K, Yazaki S, Kojima Y, Ishikawa M, Kato T, Yonemori K
Gynecol Oncol 2023 Sep;176:115-121. Epub 2023 Jul 26 doi: 10.1016/j.ygyno.2023.07.010. PMID: 37506533
Ovarian carcinosarcoma: Current developments and future perspectives.
Boussios S, Karathanasi A, Zakynthinakis-Kyriakou N, Tsiouris AK, Chatziantoniou AA, Kanellos FS, Tatsi K
Crit Rev Oncol Hematol 2019 Feb;134:46-55. Epub 2018 Dec 24 doi: 10.1016/j.critrevonc.2018.12.006. PMID: 30771873
Current management of ovarian carcinosarcoma.
Mano MS, Rosa DD, Azambuja E, Ismael G, Braga S, D'Hondt V, Piccart M, Awada A
Int J Gynecol Cancer 2007 Mar-Apr;17(2):316-24. doi: 10.1111/j.1525-1438.2006.00760.x. PMID: 17362309

Clinical prediction guides

Mesothelin expression in gynecologic carcinosarcoma: clinicopathological significance and correlation with HER2 expression.
Kitadai R, Nishikawa T, Yoshida H, Mizoguchi C, Yamamoto K, Kato T, Yonemori K
J Gynecol Oncol 2024 Mar;35(2):e11. Epub 2023 Oct 12 doi: 10.3802/jgo.2024.35.e11. PMID: 37914528Free PMC Article
Folate receptor alpha is widely expressed and a potential therapeutic target in uterine and ovarian carcinosarcoma.
Saito A, Nishikawa T, Yoshida H, Mizoguchi C, Kitadai R, Yamamoto K, Yazaki S, Kojima Y, Ishikawa M, Kato T, Yonemori K
Gynecol Oncol 2023 Sep;176:115-121. Epub 2023 Jul 26 doi: 10.1016/j.ygyno.2023.07.010. PMID: 37506533
Germline drivers of gynecologic carcinosarcomas.
Sia TY, Gordhandas SB, Birsoy O, Kemel Y, Maio A, Salo-Mullen E, Sheehan M, Hensley ML, Rubinstein M, Makker V, Grisham RN, O'Cearbhaill RE, Roche KL, Mueller JJ, Leitao MM Jr, Sonoda Y, Chi DS, Abu-Rustum NR, Berger MF, Ellenson LH, Latham A, Stadler Z, Offit K, Aghajanian C, Weigelt B, Mandelker D, Liu YL
Gynecol Oncol 2023 Jul;174:34-41. Epub 2023 May 5 doi: 10.1016/j.ygyno.2023.04.024. PMID: 37149903Free PMC Article
Imaging in gynecological disease (23): clinical and ultrasound characteristics of ovarian carcinosarcoma.
Ciccarone F, Biscione A, Moro F, Fischerova D, Savelli L, Munaretto M, Jokubkiene L, Sladkevicius P, Chiappa V, Fruscio R, Franchi D, Epstein E, Timmerman D, Froyman W, Valentin L, Scambia G, Testa AC
Ultrasound Obstet Gynecol 2022 Feb;59(2):241-247. doi: 10.1002/uog.23733. PMID: 34225386
Gynecologic Cancer InterGroup (GCIG) consensus review for uterine and ovarian carcinosarcoma.
Berton-Rigaud D, Devouassoux-Shisheboran M, Ledermann JA, Leitao MM, Powell MA, Poveda A, Beale P, Glasspool RM, Creutzberg CL, Harter P, Kim JW, Reed NS, Ray-Coquard I
Int J Gynecol Cancer 2014 Nov;24(9 Suppl 3):S55-60. doi: 10.1097/IGC.0000000000000228. PMID: 25341582

Recent systematic reviews

Chemotherapy and/or radiotherapy in combination with surgery for ovarian carcinosarcoma.
Shylasree TS, Bryant A, Athavale R
Cochrane Database Syst Rev 2013 Feb 28;2013(2):CD006246. doi: 10.1002/14651858.CD006246.pub2. PMID: 23450567Free PMC Article

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Ovarian Cancer Including Fallopian Tube Cancer and Primary Peritoneal Cancer, 2024

    Reviews

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