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Ovarian Sex Cord-Stromal Tumor, Not Otherwise Specified

MedGen UID:
1793867
•Concept ID:
C5557426
•
Neoplastic Process
Synonym: Ovarian Sex Cord-Stromal Tumor, NOS

Definition

A sex cord-stromal tumor that arises from the ovary and lacks the morphologic features that belong to the categories of granulosa-stromal tumors, Sertoli-stromal tumors, or steroid cell tumors. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Sex Cord-Stromal Tumor, Not Otherwise Specified

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

Recent clinical studies

Etiology

Detection of FOXL2 C134W Mutation Status by a Novel BaseScope In Situ Hybridization Assay is Highly Sensitive and Specific for Adult Granulosa Cell Tumors.
Hammer PM, Wang A, Beard C, Zdravkovic S, Tenney T, Liang B, Das I, Bremer R, Wang LC, McCluggage WG, Stewart CJR, Howitt BE
Mod Pathol 2023 Nov;36(11):100318. Epub 2023 Aug 25 doi: 10.1016/j.modpat.2023.100318. PMID: 37634867
Ovarian steroid cell tumors, not otherwise specified: analysis of nine cases with a literature review.
Lin M, Bao K, Lu L, Xu S, Liang Y, Cheng X, Wang F
BMC Endocr Disord 2022 Nov 1;22(1):265. doi: 10.1186/s12902-022-01170-9. PMID: 36316664Free PMC Article
Ovarian sex cord stromal tumours: analysis of the clinical and sonographic characteristics of different histopathologic subtypes.
Jiang MJ, Le Q, Yang BW, Yuan F, Chen H
J Ovarian Res 2021 Apr 17;14(1):53. doi: 10.1186/s13048-021-00805-0. PMID: 33865445Free PMC Article
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
Ovarian steroid cell tumor, not otherwise specified: a clinicopathological and immunohistochemical experience of 12 cases.
Haroon S, Idrees R, Fatima S, Memon A, Kayani N
J Obstet Gynaecol Res 2015 Mar;41(3):424-31. Epub 2014 Oct 27 doi: 10.1111/jog.12537. PMID: 25345475

Diagnosis

Virilization by an Ovarian Tumor: Presentation Is Not Always Acute.
Tjitro A, Wong DA, Ajmal A, Buddhdev K, Brady R
J Investig Med High Impact Case Rep 2022 Jan-Dec;10:23247096211056494. doi: 10.1177/23247096211056494. PMID: 35596563Free PMC Article
Malignant Sex Cord-Stromal Tumor, Not Otherwise Specified, Harboring FOXL2, p53, and TERT Promoter Mutations: Report of a Case.
Mayer R, Dandulakis M, Richards S, Roque DM, Staats PN
Int J Gynecol Pathol 2020 Nov;39(6):567-572. doi: 10.1097/PGP.0000000000000651. PMID: 31789679
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
Right ovarian steroid tumor (not otherwise specified).
Porter KK, Fradin J, Hamper UM
Ultrasound Q 2013 Sep;29(3):227-9. doi: 10.1097/RUQ.0b013e3182a1b8f8. PMID: 23945488
Magnetic resonance imaging: a valuable aid to the diagnosis of a rare ovarian tumour--steroid secreting tumour of the ovary not otherwise specified.
Annaiah TK, Webb B, Buckingham S
J Obstet Gynaecol 2010 Jan;30(1):77-8. doi: 10.3109/01443610903303021. PMID: 20121521

Therapy

Malignant Sex Cord-Stromal Tumor, Not Otherwise Specified, Harboring FOXL2, p53, and TERT Promoter Mutations: Report of a Case.
Mayer R, Dandulakis M, Richards S, Roque DM, Staats PN
Int J Gynecol Pathol 2020 Nov;39(6):567-572. doi: 10.1097/PGP.0000000000000651. PMID: 31789679
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
Patterns of metastasis in sex cord-stromal tumors of the ovary: can routine staging lymphadenectomy be omitted?
Brown J, Sood AK, Deavers MT, Milojevic L, Gershenson DM
Gynecol Oncol 2009 Apr;113(1):86-90. Epub 2009 Jan 21 doi: 10.1016/j.ygyno.2008.12.007. PMID: 19162310

Prognosis

Ovarian steroid cell tumors, not otherwise specified: analysis of nine cases with a literature review.
Lin M, Bao K, Lu L, Xu S, Liang Y, Cheng X, Wang F
BMC Endocr Disord 2022 Nov 1;22(1):265. doi: 10.1186/s12902-022-01170-9. PMID: 36316664Free PMC Article
Ovarian sex cord stromal tumours: analysis of the clinical and sonographic characteristics of different histopathologic subtypes.
Jiang MJ, Le Q, Yang BW, Yuan F, Chen H
J Ovarian Res 2021 Apr 17;14(1):53. doi: 10.1186/s13048-021-00805-0. PMID: 33865445Free PMC Article
Failure of multiple surgical procedures and adjuvant chemotherapy in early-stage steroid-cell ovarian tumor treatment: a case report and literature review.
Vasilevska D, Rudaitis V, Vasilevska D, Mickys U, Wawrysiuk S, Semczuk A
J Int Med Res 2021 Jan;49(1):300060520983195. doi: 10.1177/0300060520983195. PMID: 33435776Free PMC Article
Malignant Sex Cord-Stromal Tumor, Not Otherwise Specified, Harboring FOXL2, p53, and TERT Promoter Mutations: Report of a Case.
Mayer R, Dandulakis M, Richards S, Roque DM, Staats PN
Int J Gynecol Pathol 2020 Nov;39(6):567-572. doi: 10.1097/PGP.0000000000000651. PMID: 31789679
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391

Clinical prediction guides

A Report of a Case With Pediatric Ovarian Steroid Cell Tumor, Not Otherwise Specified, Found With Precocious Puberty.
Umeda S, Takase K, Takayama K, Yamamichi T, Higuchi K, Kawai M, Takeuchi M, Inoue M, Usui N
J Pediatr Hematol Oncol 2023 May 1;45(4):e522-e524. Epub 2022 Jun 7 doi: 10.1097/MPH.0000000000002496. PMID: 35700385
Ovarian steroid cell tumors, not otherwise specified: analysis of nine cases with a literature review.
Lin M, Bao K, Lu L, Xu S, Liang Y, Cheng X, Wang F
BMC Endocr Disord 2022 Nov 1;22(1):265. doi: 10.1186/s12902-022-01170-9. PMID: 36316664Free PMC Article
Ovarian sex cord stromal tumours: analysis of the clinical and sonographic characteristics of different histopathologic subtypes.
Jiang MJ, Le Q, Yang BW, Yuan F, Chen H
J Ovarian Res 2021 Apr 17;14(1):53. doi: 10.1186/s13048-021-00805-0. PMID: 33865445Free PMC Article
Ovarian steroid cell tumor, not otherwise specified: a clinicopathological and immunohistochemical experience of 12 cases.
Haroon S, Idrees R, Fatima S, Memon A, Kayani N
J Obstet Gynaecol Res 2015 Mar;41(3):424-31. Epub 2014 Oct 27 doi: 10.1111/jog.12537. PMID: 25345475

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