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Leydig cell tumor

MedGen UID:
9747
•Concept ID:
C0023601
•
Neoplastic Process
Synonyms: Interstitial Cell Tumor; Interstitial Cell Tumors; Leydig Cell Tumor; Tumor, Interstitial Cell; Tumor, Leydig Cell; Tumors, Interstitial Cell
 
Monarch Initiative: MONDO:0006266

Definition

A sex cord-stromal tumor occurring in the testis and rarely in the ovary. It is predominantly or completely composed of Leydig cells which may contain crystals of Reinke. In males it usually presents as a painless testicular enlargement and it may be associated with gynecomastia and decreased libido. The majority of the cases have a benign clinical course. Approximately 10% of the cases have a malignant clinical course and metastasize. In females it may be associated with androgenic manifestations and it follows a benign clinical course. [from NCI]

Professional guidelines

PubMed

Testicular adrenal rest tumors - Epidemiology, diagnosis and treatment.
Eyer de Jesus L, Paz de Oliveira AP, Porto LC, Dekermacher S
J Pediatr Urol 2024 Feb;20(1):77-87. Epub 2023 Oct 7 doi: 10.1016/j.jpurol.2023.10.005. PMID: 37845103
DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis.
Han LM, Weiel JJ, Longacre TA, Folkins AK
Adv Anat Pathol 2022 Sep 1;29(5):297-308. Epub 2022 Jul 1 doi: 10.1097/PAP.0000000000000351. PMID: 35778792
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841

Recent clinical studies

Etiology

Sertoli-Leydig cell tumor: a clinicopathological analysis in a comprehensive, national cohort.
Bekker P, Miland-Samuelsen AR, Smerdel MP, Schnack TH, Lauszus FF, Karstensen SH
Int J Gynecol Cancer 2023 Dec 4;33(12):1921-1927. doi: 10.1136/ijgc-2023-004945. PMID: 37907261
Testicular Lesions in Infertile Men.
Dupeux M, Maxwell F, Rocher L, Izard V, Guettier C, Ferlicot S
Am J Clin Pathol 2022 Jun 7;157(6):936-941. doi: 10.1093/ajcp/aqab214. PMID: 34935941
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
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
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article

Diagnosis

Sertoli-Leydig cell tumor.
Villegas Cruz C, Berlanga Narro S, Saltijeral SN, Roman Montalvo MA
Int J Gynecol Cancer 2024 Sep 2;34(9):1476-1478. doi: 10.1136/ijgc-2023-004321. PMID: 38088150
Sertoli-Leydig cell tumor: a clinicopathological analysis in a comprehensive, national cohort.
Bekker P, Miland-Samuelsen AR, Smerdel MP, Schnack TH, Lauszus FF, Karstensen SH
Int J Gynecol Cancer 2023 Dec 4;33(12):1921-1927. doi: 10.1136/ijgc-2023-004945. PMID: 37907261
New-onset hirsutism.
Proulx J, Sparling JD
J Fam Pract 2021 Apr;70(3):147-149. doi: 10.12788/jfp.0179. PMID: 34314340
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Pediatric Gynecologic Cancers.
Pommert L, Bradley W
Curr Oncol Rep 2017 Jul;19(7):44. doi: 10.1007/s11912-017-0604-7. PMID: 28501984

Therapy

INSL3 Expression in Leydig Cell Hyperplasia and Leydig Cell Tumors.
Lakis NS, Lombardo KA, Mangray S, Netto GJ, Salles D, Matoso A
Appl Immunohistochem Mol Morphol 2019 Mar;27(3):203-209. doi: 10.1097/PAI.0000000000000567. PMID: 29084059
Ovarian Sertoli-Leydig cell tumors: a single institution experience and review of the literature.
Gressel GM, Buza N, Pal L
Eur J Gynaecol Oncol 2017;38(2):214-220. PMID: 29953783
Testicular tumors of adrenogenital syndrome: From physiopathology to therapy.
Naouar S, Braiek S, El Kamel R
Presse Med 2017 Jun;46(6 Pt 1):572-578. Epub 2017 May 23 doi: 10.1016/j.lpm.2017.05.006. PMID: 28549629
Gynaecomastia--pathophysiology, diagnosis and treatment.
Narula HS, Carlson HE
Nat Rev Endocrinol 2014 Nov;10(11):684-98. Epub 2014 Aug 12 doi: 10.1038/nrendo.2014.139. PMID: 25112235
Testicular tumours.
Peckham MJ, McElwain TJ
Clin Endocrinol Metab 1975 Nov;4(3):665-92. doi: 10.1016/s0300-595x(75)80052-1. PMID: 58746

Prognosis

Sertoli-Leydig cell tumor: a clinicopathological analysis in a comprehensive, national cohort.
Bekker P, Miland-Samuelsen AR, Smerdel MP, Schnack TH, Lauszus FF, Karstensen SH
Int J Gynecol Cancer 2023 Dec 4;33(12):1921-1927. doi: 10.1136/ijgc-2023-004945. PMID: 37907261
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
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
DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.
González IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP
Mod Pathol 2022 Jan;35(1):4-22. Epub 2021 Oct 1 doi: 10.1038/s41379-021-00905-8. PMID: 34599283Free PMC Article
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841

Clinical prediction guides

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
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Imaging of DICER1 syndrome.
Guillerman RP, Foulkes WD, Priest JR
Pediatr Radiol 2019 Oct;49(11):1488-1505. Epub 2019 Oct 16 doi: 10.1007/s00247-019-04429-x. PMID: 31620849
Ovarian Sex Cord-Stromal Tumors.
Schultz KA, Harris AK, Schneider DT, Young RH, Brown J, Gershenson DM, Dehner LP, Hill DA, Messinger YH, Frazier AL
J Oncol Pract 2016 Oct;12(10):940-946. doi: 10.1200/JOP.2016.016261. PMID: 27858560Free PMC Article
The toxicology of perfluorooctanoate.
Kennedy GL Jr, Butenhoff JL, Olsen GW, O'Connor JC, Seacat AM, Perkins RG, Biegel LB, Murphy SR, Farrar DG
Crit Rev Toxicol 2004 Jul-Aug;34(4):351-84. doi: 10.1080/10408440490464705. PMID: 15328768

Recent systematic reviews

Ovarian Sertoli-Leydig cell tumours: A systematic review of relapsed cases.
Nef J, Huber DE
Eur J Obstet Gynecol Reprod Biol 2021 Aug;263:261-274. Epub 2021 Jun 28 doi: 10.1016/j.ejogrb.2021.06.036. PMID: 34245994
Testis-sparing surgery in children with testicular tumors: A systematic review and meta-analysis.
Miao X, Li Y, Zhou T, Lv M
Asian J Surg 2021 Dec;44(12):1503-1509. Epub 2021 Apr 21 doi: 10.1016/j.asjsur.2021.03.016. PMID: 33893031
Multimorbidity and Genetic Characteristics of DICER1 Syndrome Based on Systematic Review.
Cai S, Zhao W, Nie X, Abbas A, Fu L, Bihi S, Feng G, Liu T, Lv Y, Ma X, Peng X
J Pediatr Hematol Oncol 2017 Jul;39(5):355-361. doi: 10.1097/MPH.0000000000000715. PMID: 27906793
Treatment of leydig cell tumours of the testis: Can testis-sparing surgery replace radical orchidectomy? Results of a systematic review.
Bozzini G, Ratti D, Carmignani L; en representación de Young Academic Urologists (YAU) Men's Health Group
Actas Urol Esp 2017 Apr;41(3):146-154. Epub 2016 Nov 25 doi: 10.1016/j.acuro.2016.04.014. PMID: 27890492
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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