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Malignant sex cord stromal tumor of ovary

MedGen UID:
233667
•Concept ID:
C1334609
•
Neoplastic Process
Synonyms: Malignant ovarian SCST; malignant ovarian SCST; Malignant Ovarian Sex Cord-Stromal Neoplasm; malignant ovarian Sex cord-stromal neoplasm; Malignant Ovarian Sex Cord-Stromal Tumor; Malignant ovarian sex cord-stromal tumor; malignant ovarian Sex cord-stromal tumor; malignant ovarian sex cord-stromal tumor; malignant ovarian Sex cord-stromal tumour; malignant ovarian sex cord-stromal tumour; malignant sex cord stromal tumor of ovary; Malignant Sex Cord-Stromal Tumor of Ovary; malignant Sex cord-stromal tumor of ovary; Malignant Sex Cord-Stromal Tumor of the Ovary; malignant Sex cord-stromal tumor of the ovary; malignant Sex cord-stromal tumour of ovary; malignant Sex cord-stromal tumour of the ovary; ovarian sex cord-stromal tumor, malignant
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018172
Orphanet: ORPHA35808

Definition

Malignant sex cord stromal tumor (SCST) of ovary is a rare ovarian cancer (see this term) arising from granulosa, theca, sertoli and leydig cells or stromal fibroblasts, occurring at any age and presenting with abdominal or pelvic mass, and characterized (with the exception of fibroma) by the production of sex steroids resulting in manifestations of hormone excess, with a relatively favorable prognosis. [from ORDO]

Professional guidelines

PubMed

Clinical outcomes of leuprolide acetate in the treatment of recurrent ovarian granulosa cell tumors.
Foster KI, Flores Legarreta A, Kamat R, Handley KF, Glassman D, How JA, Lawson BC, Westin SN, Ramondetta LM, Frumovitz M, Gershenson DM, Sood AK, Hillman RT
Am J Obstet Gynecol 2023 Jun;228(6):724.e1-724.e9. Epub 2023 Mar 11 doi: 10.1016/j.ajog.2023.02.029. PMID: 36907533Free PMC Article
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

Sex Cord-Stromal Tumors of the Ovary.
Sertic M, Devins KM, Oliva E, Lee SI, Kilcoyne A
Radiol Clin North Am 2023 Jul;61(4):595-608. Epub 2023 Mar 31 doi: 10.1016/j.rcl.2023.02.005. PMID: 37169426
Non-Epithelial Ovarian Cancers: How Much Do We Really Know?
Cheung A, Shah S, Parker J, Soor P, Limbu A, Sheriff M, Boussios S
Int J Environ Res Public Health 2022 Jan 19;19(3) doi: 10.3390/ijerph19031106. PMID: 35162125Free PMC Article
Comprehensive review of imaging features of sex cord-stromal tumors of the ovary.
Javadi S, Ganeshan DM, Jensen CT, Iyer RB, Bhosale PR
Abdom Radiol (NY) 2021 Apr;46(4):1519-1529. Epub 2021 Mar 16 doi: 10.1007/s00261-021-02998-w. PMID: 33725145
Ovarian sex cord-stromal tumours: an update in recent molecular advances.
Lim D, Oliva E
Pathology 2018 Feb;50(2):178-189. Epub 2017 Dec 21 doi: 10.1016/j.pathol.2017.10.008. PMID: 29275930
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723

Diagnosis

Update on Ovarian Sex Cord-Stromal Tumors.
Ordulu Z
Clin Lab Med 2023 Jun;43(2):245-274. doi: 10.1016/j.cll.2023.03.001. PMID: 37169445
Sex Cord-Stromal Tumors of the Ovary.
Sertic M, Devins KM, Oliva E, Lee SI, Kilcoyne A
Radiol Clin North Am 2023 Jul;61(4):595-608. Epub 2023 Mar 31 doi: 10.1016/j.rcl.2023.02.005. PMID: 37169426
Progress in the management of ovarian granulosa cell tumor: A review.
Li J, Chu R, Chen Z, Meng J, Yao S, Song K, Kong B
Acta Obstet Gynecol Scand 2021 Oct;100(10):1771-1778. Epub 2021 Jul 16 doi: 10.1111/aogs.14189. PMID: 34027996
Natural history of Ollier disease and Maffucci syndrome: Patient survey and review of clinical literature.
El Abiad JM, Robbins SM, Cohen B, Levin AS, Valle DL, Morris CD, de Macena Sobreira NL
Am J Med Genet A 2020 May;182(5):1093-1103. Epub 2020 Mar 7 doi: 10.1002/ajmg.a.61530. PMID: 32144835Free PMC Article
Ovarian sex cord-stromal tumours and their mimics.
Young RH
Pathology 2018 Jan;50(1):5-15. Epub 2017 Nov 11 doi: 10.1016/j.pathol.2017.09.007. PMID: 29132723

Therapy

Granulosa cell tumors (GCTs) of the ovary: What is the role of radiotherapy?
Barcellini A, Mangili G, Fodor A, Secondino S, Zerbetto F, Charalampopoulou A, Pignata S, Orlandi E, Bergamini A
Crit Rev Oncol Hematol 2023 Jan;181:103889. Epub 2022 Nov 26 doi: 10.1016/j.critrevonc.2022.103889. PMID: 36503888
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
Granulosa Cell Tumor of the Ovary: A Retrospective Study of 31 Cases and a Review of the Literature.
Dridi M, Chraiet N, Batti R, Ayadi M, Mokrani A, Meddeb K, Yahiaoui Y, Raies H, Mezlini A
Int J Surg Oncol 2018;2018:4547892. Epub 2018 Mar 29 doi: 10.1155/2018/4547892. PMID: 29796312Free PMC Article
Granulosa cell tumor of the ovary: tumor review.
Koukourakis GV, Kouloulias VE, Koukourakis MJ, Zacharias GA, Papadimitriou C, Mystakidou K, Pistevou-Gompaki K, Kouvaris J, Gouliamos A
Integr Cancer Ther 2008 Sep;7(3):204-15. doi: 10.1177/1534735408322845. PMID: 18815151
Granulosa cell tumor of the ovary.
Pectasides D, Pectasides E, Psyrri A
Cancer Treat Rev 2008 Feb;34(1):1-12. Epub 2007 Oct 22 doi: 10.1016/j.ctrv.2007.08.007. PMID: 17945423

Prognosis

Update on Ovarian Sex Cord-Stromal Tumors.
Ordulu Z
Clin Lab Med 2023 Jun;43(2):245-274. doi: 10.1016/j.cll.2023.03.001. PMID: 37169445
Sex Cord-Stromal Tumors of the Ovary.
Sertic M, Devins KM, Oliva E, Lee SI, Kilcoyne A
Radiol Clin North Am 2023 Jul;61(4):595-608. Epub 2023 Mar 31 doi: 10.1016/j.rcl.2023.02.005. PMID: 37169426
Update on Ovarian Sex Cord-Stromal Tumors.
Ordulu Z
Surg Pathol Clin 2022 Jun;15(2):235-258. doi: 10.1016/j.path.2022.02.004. PMID: 35715160
Progress in the management of ovarian granulosa cell tumor: A review.
Li J, Chu R, Chen Z, Meng J, Yao S, Song K, Kong B
Acta Obstet Gynecol Scand 2021 Oct;100(10):1771-1778. Epub 2021 Jul 16 doi: 10.1111/aogs.14189. PMID: 34027996
Natural history of Ollier disease and Maffucci syndrome: Patient survey and review of clinical literature.
El Abiad JM, Robbins SM, Cohen B, Levin AS, Valle DL, Morris CD, de Macena Sobreira NL
Am J Med Genet A 2020 May;182(5):1093-1103. Epub 2020 Mar 7 doi: 10.1002/ajmg.a.61530. PMID: 32144835Free PMC Article

Clinical prediction guides

Immunohistochemical markers of prognosis in adult granulosa cell tumors of the ovary - a review.
Jung D, Almstedt K, Battista MJ, Seeger A, Jäkel J, Brenner W, Hasenburg A
J Ovarian Res 2023 Mar 3;16(1):50. doi: 10.1186/s13048-023-01125-1. PMID: 36869369Free PMC Article
Ovarian microcystic stromal tumour: from morphological observations to syndromic associations.
Parra-Herran C, McCluggage WG
Histopathology 2022 May;80(6):898-904. Epub 2022 Feb 28 doi: 10.1111/his.14616. PMID: 35020947
Imaging, clinical, and pathologic findings of Sertoli-leydig cell tumors.
Chen J, Liu Y, Zhang Y, Wang Y, Chen X, Wang Z
Sci Prog 2021 Apr-Jun;104(2):368504211009668. doi: 10.1177/00368504211009668. PMID: 33848213Free PMC Article
Ovarian tumors: a survey of selected advances of note during the life of this journal.
Young RH
Hum Pathol 2020 Jan;95:169-206. Epub 2019 Oct 22 doi: 10.1016/j.humpath.2019.09.002. PMID: 31654691
Expression of angiogenic factors in sclerosing stromal tumours of the ovary.
Hirakawa T, Kawano Y, Mizoguchi C, Nasu K, Narahara H
J Obstet Gynaecol 2018 Jul;38(5):682-685. Epub 2018 Feb 13 doi: 10.1080/01443615.2017.1397610. PMID: 29433373

Recent systematic reviews

The Prognostic Significance of Adjuvant Chemotherapy in Adult Ovarian Granulosa Cell Tumors: A Systematic Review and Meta-analysis.
Zhuang Y, Yang H
Cancer Control 2023 Jan-Dec;30:10732748231215165. doi: 10.1177/10732748231215165. PMID: 37957122Free PMC Article
Prognostic predictors in recurrent adult granulosa cell tumors of the ovary: a systematic review and meta-analysis.
Inzani F, Santoro A, Travaglino A, D'Alessandris N, Raffone A, Straccia P, Angelico G, Arciuolo D, Scaglione G, Valente M, Tralongo P, Gallotta V, Zannoni GF
Arch Gynecol Obstet 2022 Aug;306(2):315-321. Epub 2021 Nov 19 doi: 10.1007/s00404-021-06305-2. PMID: 34799743
Progress in the management of ovarian granulosa cell tumor: A review.
Li J, Chu R, Chen Z, Meng J, Yao S, Song K, Kong B
Acta Obstet Gynecol Scand 2021 Oct;100(10):1771-1778. Epub 2021 Jul 16 doi: 10.1111/aogs.14189. PMID: 34027996
Granulosa cell tumor of ovary: A systematic review of recent evidence.
Levin G, Zigron R, Haj-Yahya R, Matan LS, Rottenstreich A
Eur J Obstet Gynecol Reprod Biol 2018 Jun;225:57-61. Epub 2018 Apr 11 doi: 10.1016/j.ejogrb.2018.04.002. PMID: 29665458
Lymph-node metastasis in stage I and II sex cord stromal and malignant germ cell tumours of the ovary: a systematic review.
Kleppe M, Amkreutz LC, Van Gorp T, Slangen BF, Kruse AJ, Kruitwagen RF
Gynecol Oncol 2014 Apr;133(1):124-7. Epub 2014 Jan 16 doi: 10.1016/j.ygyno.2014.01.011. PMID: 24440833

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