Malignant Leydig cell tumor

MedGen UID:: 83143
•Concept ID:: C0334410
•: Neoplastic Process

Synonyms:	cancer of Leydig cell; Interstitial cell tumor, malignant; Leydig cell cancer; Leydig cell tumor, malignant; Malignant Interstitial Cell Neoplasm; malignant interstitial cell neoplasm; Malignant interstitial cell tumor; Malignant Interstitial Cell Tumor; malignant interstitial cell tumor; malignant interstitial cell tumour; Malignant Leydig Cell Neoplasm; malignant Leydig cell neoplasm; Malignant Leydig Cell Tumor; malignant Leydig cell tumor; malignant neoplasm of Leydig cell
SNOMED CT:	Malignant interstitial cell tumor (77870005); Malignant Leydig cell tumor (77870005); Leydig cell tumor, malignant (77870005); Interstitial cell tumor, malignant (77870005)

Monarch Initiative:	MONDO:0000377

Definition

A Leydig cell tumor characterized by large tumor size, the presence of cytologic atypia, increased mitotic activity, necrosis, and vascular invasion. Approximately 10% of the testicular Leydig cell tumors show malignant characteristics and metastasize. Leydig cell tumors of the ovary follow a benign clinical course. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVMalignant Leydig cell tumor

Disorder of female genital system
- Adnexal Diseases
  - Disorder of ovary
    - Ovarian neoplasm
      - Sertoli-Leydig cell tumor
        Leydig cell tumor
        Malignant Leydig cell tumor
        Malignant testicular Leydig cell tumor

Professional guidelines

PubMed

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

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

Diagnosis and management of hirsute women.

Badawy SZ
Int J Fertil 1987 Sep-Oct;32(5):349-57. PMID: 2889680

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Recent clinical studies

Etiology

Retroperitoneal Leydig cell tumor recurrence presenting 14 years after orchiectomy.

Chakiryan NH, Raess PW, Turner K, Hedges JC, Liu JJ
Can J Urol 2018 Feb;25(1):9210-9213. PMID: 29524977

See all (1)

Diagnosis

Utilization of NKX3.1, P501S, Prostate-Specific Antigen, and Steroidogenic Factor 1 to Distinguish Malignant Leydig Cell Tumor From Metastatic Prostatic Adenocarcinoma to the Testis.

Erak E, Ulbright TM, Epstein J
Arch Pathol Lab Med 2023 Dec 1;147(12):1458-1460. doi: 10.5858/arpa.2022-0424-OA. PMID: 36881770

Malignant Leydig Cell Tumor in Elderly Complete Androgen Insensitivity Patient: A Case Report.

Shrestha S, Banepali N, Sthapit R, Agrawal D
JNMA J Nepal Med Assoc 2019 Mar-Apr;57(216):127-129. doi: 10.31729/jnma.4206. PMID: 31477948 Free PMC Article

Fine needle aspiration diagnosis of metastatic Leydig cell tumor. Report of a case and review of the literature.

Biemer J, Pambuccian SE, Barkan GA
J Am Soc Cytopathol 2019 Jul-Aug;8(4):220-229. Epub 2019 Feb 16 doi: 10.1016/j.jasc.2019.02.001. PMID: 31272604

A malignant Leydig cell tumor of the testis.

Sugimoto K, Matsumoto S, Nose K, Kurita T, Uemura H, Park YC, Hanai J
Int Urol Nephrol 2006;38(2):291-2. doi: 10.1007/s11255-006-6661-8. PMID: 16868700

Leydig cell tumor in an elderly patient with complete androgen insensitivity syndrome.

Iwamoto I, Yanazume S, Fujino T, Yoshioka T, Douchi T
Gynecol Oncol 2005 Mar;96(3):870-2. doi: 10.1016/j.ygyno.2004.11.015. PMID: 15721441

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Therapy

A patient with ectopic cortisol production derived from malignant testicular masses.

Jain SH, Sadow PM, Nosé V, Dluhy RG
Nat Clin Pract Endocrinol Metab 2008 Dec;4(12):695-700. Epub 2008 Oct 21 doi: 10.1038/ncpendmet0985. PMID: 18941436

Lack of efficacy of imatinib in a patient with metastatic Leydig cell tumor.

Froehner M, Beuthien-Baumann B, Dittert DD, Schuler U, Wirth MP
Cancer Chemother Pharmacol 2006 Nov;58(5):716-8. Epub 2006 Feb 1 doi: 10.1007/s00280-005-0181-6. PMID: 16450163

Malignant Leydig cell tumor of the testis in complete remission on o,p'-dichlorodiphenyl-dichloroethane.

van der Hem KG, Boven E, van Hennik MB, Pinedo HM
J Urol 1992 Oct;148(4):1256-9. doi: 10.1016/s0022-5347(17)36878-7. PMID: 1404649

Treatment of malignant Leydig cell tumor.

Bertram KA, Bratloff B, Hodges GF, Davidson H
Cancer 1991 Nov 15;68(10):2324-9. doi: 10.1002/1097-0142(19911115)68:10<2324::aid-cncr2820681036>3.0.co;2-k. PMID: 1913469

Malignant Leydig cell tumor: objective tumor response to o,p'-DDD.

Azer PC, Braunstein GD
Cancer 1981 Mar 15;47(6):1251-5. doi: 10.1002/1097-0142(19810315)47:6<1251::aid-cncr2820470604>3.0.co;2-r. PMID: 7226052

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