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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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVOvarian sex cord-stromal tumor

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Neoplasm
      - Ovarian sex cord-stromal tumor
        Benign ovarian sex cord-stromal tumor
        Benign Ovarian Thecoma
        Benign Ovarian Luteinized Thecoma
        Typical Ovarian Thecoma
        Fibroma of ovary
        Ovarian Cellular Fibroma
        Ovarian microcystic stromal tumor
        Ovarian Sclerosing Stromal Tumor
        Ovarian Stromal-Leydig Cell Tumor
        Juvenile type ovarian granulosa cell tumor
        Juvenile Bilateral Ovarian Granulosa Cell Tumor
        Malignant sex cord stromal tumor of ovary
        Maligant granulosa cell tumor of ovary
        Recurrent Malignant Ovarian Granulosa Cell Tumor
        Malignant Ovarian Steroid Cell Tumor
        Malignant Ovarian Thecoma
        Malignant Ovarian Luteinized Thecoma
        Malignant Sertoli-Leydig cell tumor of ovary
        Ovarian gynandroblastoma
        Poorly Differentiated Ovarian Sertoli-Leydig Cell Tumor
        Poorly Differentiated Ovarian Sertoli-Leydig Cell Tumor, Variant with Heterologous Elements
        Theca steroid-producing cell malignant tumor of ovary, not further specified
        Ovarian Granulosa-Stromal Cell Tumor
        Ovarian granulosa cell tumor
        Adult Ovarian Granulosa Cell Tumor
        Ovarian Tumor of the Thecoma/Fibroma Group
        Ovarian Signet Ring Stromal Tumor
        Ovarian thecoma
        Ovarian sertoli-stromal cell tumor
        Ovarian Sertoli Cell Tumor
        Ovarian Sertoli Cell Tumor with Lipid Storage
        Ovarian Sertoli-Leydig cell tumor
        Moderately Differentiated Ovarian Sertoli-Leydig Cell Tumor
        Ovarian Retiform Sertoli-Leydig Cell Tumor
        Ovarian Sertoli-Leydig Cell Tumor with Heterologous Elements
        Ovarian Sertoli-Leydig Cell Tumor with Retiform Elements
        Well Differentiated Ovarian Sertoli-Leydig Cell Tumor
        Ovarian Sex Cord Tumor with Annular Tubules
        Ovarian Sex Cord-Stromal Tumor Associated with Peutz-Jeghers Syndrome
        Ovarian Sex Cord-Stromal Tumor, Not Otherwise Specified
        Ovarian Steroid Cell Tumor
        Ovarian Leydig Cell Tumor, Not Otherwise Specified
        Ovarian Hilus Cell Tumor
        Ovarian Leydig Cell Tumor, Non-Hilar Type
        Ovarian Steroid Cell Tumor, Not Otherwise Specified
        Well Differentiated Ovarian Steroid Cell Tumor
        Ovarian Stromal Luteoma
        Ovarian Stromal Tumor with Minor Sex Cord Elements

Professional guidelines

PubMed

Comment on: Consensus recommendations from the EXPeRT/PARTNER groups for the diagnosis and therapy of sex cord stromal tumors in children and adolescents.

de Faria FW, Valera ET, Macedo CRPD, Azevedo EF, Vieira AGS, Martins GE, da Cunha Júnior AG, Francisco Dos Reis MB, Foulkes WD, Lopes LF
Pediatr Blood Cancer 2022 Sep;69(9):e29650. Epub 2022 Mar 26 doi: 10.1002/pbc.29650. PMID: 35338687

Non-epithelial ovarian cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.

Colombo N, Peiretti M, Garbi A, Carinelli S, Marini C, Sessa C; ESMO Guidelines Working Group
Ann Oncol 2012 Oct;23 Suppl 7:vii20-6. doi: 10.1093/annonc/mds223. PMID: 22997450

Ovarian sex cord-stromal tumors. Problems in differential diagnosis.

Young RH, Scully RE
Pathol Annu 1988;23 Pt 1:237-96. PMID: 2838794

See all (107)

Recent clinical studies

Etiology

FOXL2 Mutation Status in Sex Cord-stromal Tumors Cannot be Predicted by Morphology.

Wessman S, Fuentes BB, Severin-Karlsson J, Westbom-Fremer S, Nistér M, Kokaraki G, Petta TB, Haglund F, Carlson JW
Int J Gynecol Pathol 2024 Jan 1;43(1):78-89. Epub 2023 Apr 10 doi: 10.1097/PGP.0000000000000953. PMID: 37255476

BAP1 Tumor Predisposition Syndrome Presenting as a Recurrent Ovarian Sex Cord-Stromal Tumor.

Albright BB, Wignall E, Bentley RC, Havrilesky LJ, Previs RA, Strickland KC
Int J Gynecol Pathol 2023 Jan 1;42(1):83-88. Epub 2022 May 20 doi: 10.1097/PGP.0000000000000855. PMID: 35348477 Free PMC Article

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

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

See all (13)

Diagnosis

FOXL2 Mutation Status in Sex Cord-stromal Tumors Cannot be Predicted by Morphology.

Juvenile granulosa cell tumor of the ovary: A comprehensive clinicopathologic analysis of 15 cases.

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.

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

See all (33)

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

See all (3)

Prognosis

FOXL2 Mutation Status in Sex Cord-stromal Tumors Cannot be Predicted by Morphology.

Juvenile granulosa cell tumor of the ovary: A comprehensive clinicopathologic analysis of 15 cases.

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: 32943602 Free 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

See all (12)

Clinical prediction guides

FOXL2 Mutation Status in Sex Cord-stromal Tumors Cannot be Predicted by Morphology.

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

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: 32943602 Free PMC Article

Pediatric imaging in DICER1 syndrome.

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

See all (9)

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

See all (1)

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Ovarian sex cord-stromal tumor

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

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Clinical resources

Practice guidelines

Consumer resources

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