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Ovarian sex cord-stromal tumor

MedGen UID:
154644
•Concept ID:
C0600113
•
Neoplastic Process
Synonym: Ovarian sex cord tumors
SNOMED CT: Stromal tumor of ovary (237058000); Sex cord tumor of ovary (237058000)
 
HPO: HP:0031918
Monarch Initiative: MONDO:0021657

Definition

A benign or malignant neoplasm that arises from the ovary and is composed of granulosa cells, Sertoli cells, Leydig cells, theca cells, and fibroblasts. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian sex cord-stromal tumor

Professional guidelines

PubMed

Ovarian sex cord-stromal tumors: an update on clinical features, molecular changes, and management.
Al Harbi R, McNeish IA, El-Bahrawy M
Int J Gynecol Cancer 2021 Feb;31(2):161-168. Epub 2021 Jan 7 doi: 10.1136/ijgc-2020-002018. PMID: 33414107
Rare ovarian tumors: an update on diagnosis and treatment.
Debuquoy C, Romeo C, Vanacker H, Ray-Coquard I
Int J Gynecol Cancer 2020 Jun;30(6):879-887. Epub 2020 May 26 doi: 10.1136/ijgc-2020-001235. PMID: 32461259
Ovarian collision tumors: imaging findings, pathological characteristics, diagnosis, and differential diagnosis.
Peng Y, Lin J, Guan J, Chen L, Zhang X, Li S, Wang H, Liu M, Guo Y
Abdom Radiol (NY) 2018 Aug;43(8):2156-2168. doi: 10.1007/s00261-017-1419-6. PMID: 29198011

Recent clinical studies

Etiology

Juvenile granulosa cell tumor of the ovary: A comprehensive clinicopathologic analysis of 15 cases.
Parikshaa G, Ariba Z, Pranab D, Nalini G, Manish R, Vanita S, Bhavana R, Devi D, Ram S, Prema M, Arvind R, Radhika S
Ann Diagn Pathol 2021 Jun;52:151721. Epub 2021 Feb 10 doi: 10.1016/j.anndiagpath.2021.151721. PMID: 33725665
Development and Validation of a Prognostic Prediction Model for Postoperative Ovarian Sex Cord-Stromal Tumor Patients.
You D, Zhang Z, Cao M
Med Sci Monit 2020 Sep 18;26:e925844. doi: 10.12659/MSM.925844. PMID: 32943602Free PMC Article
Prognostic significance of lymphadenectomyin malignant ovarian sex cord stromal tumor: A retrospective cohort study and meta-analysis.
Cheng H, Peng J, Yang Z, Zhang G
Gynecol Oncol 2018 Jan;148(1):91-96. Epub 2017 Nov 6 doi: 10.1016/j.ygyno.2017.10.022. PMID: 29107349
Pediatric imaging in DICER1 syndrome.
Bueno MT, Martínez-Ríos C, la Puente Gregorio A, Ahyad RA, Villani A, Druker H, van Engelen K, Gallinger B, Aronoff L, Grant R, Malkin D, Greer MC
Pediatr Radiol 2017 Sep;47(10):1292-1301. Epub 2017 May 4 doi: 10.1007/s00247-017-3875-0. PMID: 28474256
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature.
Blake EA, Carter CM, Kashani BN, Kodama M, Mabuchi S, Yoshino K, Matsuo K
Eur J Obstet Gynecol Reprod Biol 2014 Apr;175:1-7. Epub 2013 Dec 25 doi: 10.1016/j.ejogrb.2013.12.025. PMID: 24439718

Diagnosis

Juvenile granulosa cell tumor of the ovary: A comprehensive clinicopathologic analysis of 15 cases.
Parikshaa G, Ariba Z, Pranab D, Nalini G, Manish R, Vanita S, Bhavana R, Devi D, Ram S, Prema M, Arvind R, Radhika S
Ann Diagn Pathol 2021 Jun;52:151721. Epub 2021 Feb 10 doi: 10.1016/j.anndiagpath.2021.151721. PMID: 33725665
Ovarian Sex Cord Stromal Tumor, Steroid Cell, NOS in an Adolescent: A Case Report.
Schnuckle EM, Williamson A, Carpentieri D, Taylor S
J Pediatr Adolesc Gynecol 2021 Feb;34(1):94-97. Epub 2020 Aug 8 doi: 10.1016/j.jpag.2020.08.001. PMID: 32781238
Ovarian Sex Cord-stromal Tumors With Melanin Pigment: Report of a Previously Undescribed Phenomenon.
Taylor J, McCluggage WG
Int J Gynecol Pathol 2019 Jan;38(1):92-96. doi: 10.1097/PGP.0000000000000471. PMID: 29140884
Pediatric imaging in DICER1 syndrome.
Bueno MT, Martínez-Ríos C, la Puente Gregorio A, Ahyad RA, Villani A, Druker H, van Engelen K, Gallinger B, Aronoff L, Grant R, Malkin D, Greer MC
Pediatr Radiol 2017 Sep;47(10):1292-1301. Epub 2017 May 4 doi: 10.1007/s00247-017-3875-0. PMID: 28474256
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature.
Blake EA, Carter CM, Kashani BN, Kodama M, Mabuchi S, Yoshino K, Matsuo K
Eur J Obstet Gynecol Reprod Biol 2014 Apr;175:1-7. Epub 2013 Dec 25 doi: 10.1016/j.ejogrb.2013.12.025. PMID: 24439718

Therapy

The association of reproductive factors with risk of non-epithelial ovarian cancer and comparison with serous ovarian cancer.
Hemmingsen CH, Kjaer SK, Bennetsen AKK, Dehlendorff C, Baandrup L
Gynecol Oncol 2021 Aug;162(2):469-474. Epub 2021 May 18 doi: 10.1016/j.ygyno.2021.05.008. PMID: 34016454
Prognostic significance of lymphadenectomyin malignant ovarian sex cord stromal tumor: A retrospective cohort study and meta-analysis.
Cheng H, Peng J, Yang Z, Zhang G
Gynecol Oncol 2018 Jan;148(1):91-96. Epub 2017 Nov 6 doi: 10.1016/j.ygyno.2017.10.022. PMID: 29107349
Androgen-secreting steroid cell tumor of the ovary.
Powell JL, Dulaney DP, Shiro BC
South Med J 2000 Dec;93(12):1201-4. PMID: 11142457

Prognosis

Juvenile granulosa cell tumor of the ovary: A comprehensive clinicopathologic analysis of 15 cases.
Parikshaa G, Ariba Z, Pranab D, Nalini G, Manish R, Vanita S, Bhavana R, Devi D, Ram S, Prema M, Arvind R, Radhika S
Ann Diagn Pathol 2021 Jun;52:151721. Epub 2021 Feb 10 doi: 10.1016/j.anndiagpath.2021.151721. PMID: 33725665
The role of FOXL2, SOX9, and β-catenin expression and DICER1 mutation in differentiating sex cord tumor with annular tubules from other sex cord tumors of the ovary.
Onder S, Hurdogan O, Bayram A, Yilmaz I, Sozen H, Yavuz E
Virchows Arch 2021 Aug;479(2):317-324. Epub 2021 Feb 10 doi: 10.1007/s00428-021-03052-2. PMID: 33566167
Development and Validation of a Prognostic Prediction Model for Postoperative Ovarian Sex Cord-Stromal Tumor Patients.
You D, Zhang Z, Cao M
Med Sci Monit 2020 Sep 18;26:e925844. doi: 10.12659/MSM.925844. PMID: 32943602Free PMC Article
Non-epithelial ovarian cancer in Denmark - Incidence and survival over nearly 40 years.
Bennetsen AKK, Baandrup L, Aalborg GL, Kjaer SK
Gynecol Oncol 2020 Jun;157(3):693-699. Epub 2020 Mar 27 doi: 10.1016/j.ygyno.2020.03.021. PMID: 32223987
Prognostic significance of lymphadenectomyin malignant ovarian sex cord stromal tumor: A retrospective cohort study and meta-analysis.
Cheng H, Peng J, Yang Z, Zhang G
Gynecol Oncol 2018 Jan;148(1):91-96. Epub 2017 Nov 6 doi: 10.1016/j.ygyno.2017.10.022. PMID: 29107349

Clinical prediction guides

Well-differentiated Sertoli-Leydig Cell Tumors (SLCTs) Are Not Associated With DICER1 Pathogenic Variants and Represent a Different Tumor Type to Moderately and Poorly Differentiated SLCTs.
McCluggage WG, Rivera B, Chong AS, Clarke BA, Schultz KAP, Dehner LP, Tchrakian N, Apellaniz-Ruiz M, Gilks CB, Kommoss F, Stewart CJR, Foulkes WD
Am J Surg Pathol 2023 Apr 1;47(4):490-496. Epub 2022 Dec 30 doi: 10.1097/PAS.0000000000002010. PMID: 36583307
The role of FOXL2, SOX9, and β-catenin expression and DICER1 mutation in differentiating sex cord tumor with annular tubules from other sex cord tumors of the ovary.
Onder S, Hurdogan O, Bayram A, Yilmaz I, Sozen H, Yavuz E
Virchows Arch 2021 Aug;479(2):317-324. Epub 2021 Feb 10 doi: 10.1007/s00428-021-03052-2. PMID: 33566167
Development and Validation of a Prognostic Prediction Model for Postoperative Ovarian Sex Cord-Stromal Tumor Patients.
You D, Zhang Z, Cao M
Med Sci Monit 2020 Sep 18;26:e925844. doi: 10.12659/MSM.925844. PMID: 32943602Free PMC Article
Pediatric imaging in DICER1 syndrome.
Bueno MT, Martínez-Ríos C, la Puente Gregorio A, Ahyad RA, Villani A, Druker H, van Engelen K, Gallinger B, Aronoff L, Grant R, Malkin D, Greer MC
Pediatr Radiol 2017 Sep;47(10):1292-1301. Epub 2017 May 4 doi: 10.1007/s00247-017-3875-0. PMID: 28474256
Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature.
Blake EA, Carter CM, Kashani BN, Kodama M, Mabuchi S, Yoshino K, Matsuo K
Eur J Obstet Gynecol Reprod Biol 2014 Apr;175:1-7. Epub 2013 Dec 25 doi: 10.1016/j.ejogrb.2013.12.025. PMID: 24439718

Recent systematic reviews

Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: a systematic review of literature.
Blake EA, Carter CM, Kashani BN, Kodama M, Mabuchi S, Yoshino K, Matsuo K
Eur J Obstet Gynecol Reprod Biol 2014 Apr;175:1-7. Epub 2013 Dec 25 doi: 10.1016/j.ejogrb.2013.12.025. PMID: 24439718

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