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Ovarian Retiform Sertoli-Leydig Cell Tumor

MedGen UID:
309513
•Concept ID:
C1518728
•
Neoplastic Process

Definition

A Sertoli-Leydig cell tumor of the ovary in which anastomosing, slit-like spaces resembling rete testis constitute at least 90% of the tumor elements. It may present with estrogenic and less often androgenic manifestations. It usually follows a benign clinical course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Retiform Sertoli-Leydig Cell Tumor

Recent clinical studies

Etiology

Reclassification of two germline DICER1 splicing variants leads to DICER1 syndrome diagnosis.
Apellaniz-Ruiz M, Sabbaghian N, Chong AL, de Kock L, Cetinkaya S, Bayramoğlu E, Dinjens WNM, McCluggage WG, Wagner A, Yilmaz AA, Foulkes WD
Fam Cancer 2023 Oct;22(4):487-493. Epub 2023 May 30 doi: 10.1007/s10689-023-00336-1. PMID: 37248399Free PMC Article
Characteristics and outcomes analysis of ovarian Sertoli-Leydig cell tumors (SLCTs): analysis of 15 patients.
Wang G, Zhang R, Li C, Chen A
J Ovarian Res 2021 Nov 4;14(1):150. doi: 10.1186/s13048-021-00909-7. PMID: 34736487Free PMC Article
Ovarian Sertoli-Leydig cell tumors: an analysis of 13 cases.
Guo Y, Wang J, Li Y, Wang Y
Arch Gynecol Obstet 2020 Jul;302(1):203-208. Epub 2020 May 19 doi: 10.1007/s00404-020-05588-1. PMID: 32430758
Sertoli-Leydig cell tumor of the ovary: Analysis of a single institution database and review of the literature.
Durmuş Y, Kılıç Ç, Çakır C, Yüksel D, Boran N, Karalök A, Boyraz G, Turan AT
J Obstet Gynaecol Res 2019 Jul;45(7):1311-1318. Epub 2019 May 20 doi: 10.1111/jog.13977. PMID: 31106943
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

Reclassification of two germline DICER1 splicing variants leads to DICER1 syndrome diagnosis.
Apellaniz-Ruiz M, Sabbaghian N, Chong AL, de Kock L, Cetinkaya S, Bayramoğlu E, Dinjens WNM, McCluggage WG, Wagner A, Yilmaz AA, Foulkes WD
Fam Cancer 2023 Oct;22(4):487-493. Epub 2023 May 30 doi: 10.1007/s10689-023-00336-1. PMID: 37248399Free PMC Article
Ovarian Sertoli-Leydig cell tumors: an analysis of 13 cases.
Guo Y, Wang J, Li Y, Wang Y
Arch Gynecol Obstet 2020 Jul;302(1):203-208. Epub 2020 May 19 doi: 10.1007/s00404-020-05588-1. PMID: 32430758
Ovarian Sertoli cell tumours: practical points.
Gheorghisan-Galateanu AA, Carsote M, Terzea D, Valea A, Ghemigian A
J Pak Med Assoc 2020 Jan;70(1):129-133. doi: 10.5455/JPMA.1381. PMID: 31954037
Sertoli-Leydig cell tumor of the ovary: Analysis of a single institution database and review of the literature.
Durmuş Y, Kılıç Ç, Çakır C, Yüksel D, Boran N, Karalök A, Boyraz G, Turan AT
J Obstet Gynaecol Res 2019 Jul;45(7):1311-1318. Epub 2019 May 20 doi: 10.1111/jog.13977. PMID: 31106943
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

Characteristics and outcomes analysis of ovarian Sertoli-Leydig cell tumors (SLCTs): analysis of 15 patients.
Wang G, Zhang R, Li C, Chen A
J Ovarian Res 2021 Nov 4;14(1):150. doi: 10.1186/s13048-021-00909-7. PMID: 34736487Free PMC Article
Results from a Monocentric Long-Term Analysis of 23 Patients with Ovarian Sertoli-Leydig Cell Tumors.
Gouy S, Arfi A, Maulard A, Pautier P, Bentivegna E, Leary A, Chargari C, Genestie C, Morice P
Oncologist 2019 May;24(5):702-709. Epub 2018 Sep 10 doi: 10.1634/theoncologist.2017-0632. PMID: 30201740Free PMC Article
A clinicopathological analysis of 40 cases of ovarian Sertoli-Leydig cell tumors.
Gui T, Cao D, Shen K, Yang J, Zhang Y, Yu Q, Wan X, Xiang Y, Xiao Y, Guo L
Gynecol Oncol 2012 Nov;127(2):384-9. Epub 2012 Jul 28 doi: 10.1016/j.ygyno.2012.07.114. PMID: 22850410
Pure cystic nephroblastoma of the ovary with a review of extrarenal Wilms' tumors.
Isaac MA, Vijayalakshmi S, Madhu CS, Bosincu L, Nogales FF
Hum Pathol 2000 Jun;31(6):761-4. doi: 10.1053/hupa.2000.7627. PMID: 10872672

Prognosis

Reclassification of two germline DICER1 splicing variants leads to DICER1 syndrome diagnosis.
Apellaniz-Ruiz M, Sabbaghian N, Chong AL, de Kock L, Cetinkaya S, Bayramoğlu E, Dinjens WNM, McCluggage WG, Wagner A, Yilmaz AA, Foulkes WD
Fam Cancer 2023 Oct;22(4):487-493. Epub 2023 May 30 doi: 10.1007/s10689-023-00336-1. PMID: 37248399Free PMC Article
Characteristics and outcomes analysis of ovarian Sertoli-Leydig cell tumors (SLCTs): analysis of 15 patients.
Wang G, Zhang R, Li C, Chen A
J Ovarian Res 2021 Nov 4;14(1):150. doi: 10.1186/s13048-021-00909-7. PMID: 34736487Free PMC Article
Ovarian Sertoli-Leydig cell tumors: an analysis of 13 cases.
Guo Y, Wang J, Li Y, Wang Y
Arch Gynecol Obstet 2020 Jul;302(1):203-208. Epub 2020 May 19 doi: 10.1007/s00404-020-05588-1. PMID: 32430758
Sertoli - Leydig cell tumor with retiform areas and overgrowth of rhabdomyosarcomatous elements: case report and literature review.
Burnik Papler T, Frković Grazio S, Kobal B
J Ovarian Res 2016 Jul 30;9(1):46. doi: 10.1186/s13048-016-0257-4. PMID: 27473538Free PMC Article
Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases.
Young RH, Scully RE
Am J Surg Pathol 1985 Aug;9(8):543-69. doi: 10.1097/00000478-198508000-00001. PMID: 3911780

Clinical prediction guides

Reclassification of two germline DICER1 splicing variants leads to DICER1 syndrome diagnosis.
Apellaniz-Ruiz M, Sabbaghian N, Chong AL, de Kock L, Cetinkaya S, Bayramoğlu E, Dinjens WNM, McCluggage WG, Wagner A, Yilmaz AA, Foulkes WD
Fam Cancer 2023 Oct;22(4):487-493. Epub 2023 May 30 doi: 10.1007/s10689-023-00336-1. PMID: 37248399Free PMC Article
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
Characteristics and outcomes analysis of ovarian Sertoli-Leydig cell tumors (SLCTs): analysis of 15 patients.
Wang G, Zhang R, Li C, Chen A
J Ovarian Res 2021 Nov 4;14(1):150. doi: 10.1186/s13048-021-00909-7. PMID: 34736487Free 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
Sertoli - Leydig cell tumor with retiform areas and overgrowth of rhabdomyosarcomatous elements: case report and literature review.
Burnik Papler T, Frković Grazio S, Kobal B
J Ovarian Res 2016 Jul 30;9(1):46. doi: 10.1186/s13048-016-0257-4. PMID: 27473538Free PMC Article

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