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Disease Profile

Sertoli-leydig cell tumors

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.


US Estimated

Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

Arrhenoblastoma; Androblastoma of ovary; Sertoli-leydig cell tumor of the ovary;


Rare Cancers


Sertoli-Leydig cell tumors are a cancer that starts in the female ovaries. The cancer cells produce and release a male sex hormone which may cause the development of male physical characteristics (virilization), including facial hair and a deep voice.[1] This type of tumor is sometimes called arrhenoblastoma of the ovary or a stromal tumor.[1][2] Sertoli-Leydig cell tumors are rare tumors which account for less than 0.5% of all ovarian tumors. While they can be found in women of all age groups, they are most common in young women. Treatment involves surgery and chemotherapy or radiation if the cancer has spread. Because Sertoli-Leydig cell tumors have a low chance of spreading, if the tumor is found early, chances for survival are good.[2]


Because Sertoli-Leydig cell tumors of the ovary are rare, there are no established guidelines for treatment. Treatment decisions are based on the unique characteristics of each tumor, particularly the stage. Surgery to remove the tumor is considered the best treatment for an early-stage tumor, whereas surgery and chemotherapy may be needed to treat a later-stage tumor.[5][6]


Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Organizations Providing General Support

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
        • PubMed is a searchable database of medical literature and lists journal articles that discuss Sertoli-leydig cell tumors. Click on the link to view a sample search on this topic.


          1. Dugdale DC, Mason JR. Sertoli-Leydig cell tumor. MedlinePlus. 2008; https://www.nlm.nih.gov/medlineplus/ency/article/001172.htm. Accessed 3/25/2010.
          2. Vorvick LJ, Chen YB. Arrhenoblastoma of ovary. MedlinePlus. 2010; https://www.nlm.nih.gov/medlineplus/ency/article/001507.htm. Accessed 3/25/2010.
          3. Slade I, Bacchelli C, Davies H, Murray A, Abbaszadeh F, Hanks S, Barfoot R, Burke A, Chisholm J, Hewitt M, Jenkinson H, King D, Morland B, Pizer B, Prescott K, Saggar A, Side L, Traunecker H, Vaidya S, Ward P, Futreal PA, Vujanic G, Nicholson AG, Sebire N, Turnbull C, Priest JR, Pritchard-Jones K, Houlston R, Stiller C, Stratton MR, Douglas J, Rahman N. DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome. Journal of Medical Genetics. 2011; 48:273-278. https://www.ncbi.nlm.nih.gov/pubmed/21266384. Accessed 1/1/2013.
          4. Rio Frio T, Bahubeshi A, Kanellopoulou C, Hamel N, Niedziela M, Sabbaghian N, Pouchet C, Gilbert L, O'Brien PK, Serfas K, Broderick P, Houlston RS, Lesueur F, Bonora E, Muljo S, Schimke RN, Bouron-Dal Soglio D, Arseneau J, Schultz KA, Priest JR, Nguyen VH, Harach HR, Livingston DM, Foulkes WD, Tischkowitz M. DICER1 mutations in familial multinodular goiter with and without ovarian Sertoli-Leydig cell tumors. JAMA. 2011; 305:68-77. https://www.ncbi.nlm.nih.gov/pubmed/21205968. Accessed 1/1/2013.
          5. Gui T, Cao D, Shen K, Yang J, Zhang Y, Yu Q, Wan X, Xiang Y, Xiao Y, Guo L. A clinicopathological analysis of 40 cases of ovarian Sertoli-Leydig cell tumors. Gynecologic Oncology. 2012; 127:384-389. https://www.ncbi.nlm.nih.gov/pubmed/22850410. Accessed 1/1/2013.
          6. Sigismondi C, Gadducci A, Lorusso D, Candiani M, Breda E, Raspagliesi F, Cormio G, Marinaccio M, Mangili G. Ovarian Sertoli-Leydig cell tumors. a retrospective MITO study. Gynecologic Oncology. 2012; 125:673-676. https://www.ncbi.nlm.nih.gov/pubmed/22446621. Accessed 1/1/2013.

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