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Genetic and Rare Diseases Information Center (GARD)

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Sertoli-leydig cell tumors


Other Names for this Disease
  • Androblastoma of ovary
  • Arrhenoblastoma
  • Arrhenoblastoma of ovary
  • Sertoli-leydig cell tumor of the ovary
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Your Question

My 15 year old granddaughter has been diagnosed with a Sertoli-leydig cell tumor in her ovary.  What are her chances of survival if it is a cancerous tumor?  She also has nodules on her thyroid, and the doctors are planning to remove her thyroid.  Her mother had the same thing, but it is in remission.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is the chance of survival in an individual diagnosed with a Sertoli-Leydig cell tumor?

The chance of surviving a Sertoli-Leydig cell tumor of the ovary is quite high.  The outlook is most favorable for individuals diagnosed at an early stage, meaning the cancerous cells have not spread into nearby healthy organs.[1][2]  One study showed that 92% of individuals diagnosed with a Sertoli-Leydig cell tumor at an early stage were alive five years after their diagnosis compared to 33% of individuals with tumors diagnosed at later stages.[2]
Last updated: 1/1/2013

How might a Sertoli-Leydig cell tumor of the ovary be treated?

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.[1][2]
Last updated: 1/1/2013

Is Sertoli-Leydig cell tumor of the ovary associated with thyroid nodules?

Several articles in the medical literature have described an association between Sertoli-Leydig cell tumor of the ovary and thyroid nodules.[3][4]  It is thought that they may be related through a genetic predisposition, namely a mutation in the DICER1 gene.[4]
Last updated: 1/1/2013

Can Sertoli-Leydig cell tumor of the ovary be inherited?

There is evidence that some Sertoli-Leydig cell tumors of the ovary might be caused by a genetic predisposition to develop cancer.  Mutations in the DICER1 gene have been associated with an increased chance to develop Sertoli-Leydig cell tumors of the ovary, as well as other types of cancer.[5]  It appears that mutations in DICER1 can be passed from a parent to a child in an autosomal dominant manner.[4]
Last updated: 1/1/2013

References
  • 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. http://www.ncbi.nlm.nih.gov/pubmed/22850410. Accessed 1/1/2013.
  • 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. http://www.ncbi.nlm.nih.gov/pubmed/22446621. Accessed 1/1/2013.
  • Chi M, Gilman AD, Iroegbu N. Management of metastatic ovarian Sertoli-Leydig cell tumor with sporadic multinodular goiter: a case report and literature review. Future Oncology. 2011; 7:1113-1117. http://www.ncbi.nlm.nih.gov/pubmed/21919698. Accessed 1/1/2013.
  • 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. http://www.ncbi.nlm.nih.gov/pubmed/21205968. Accessed 1/1/2013.
  • 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. http://www.ncbi.nlm.nih.gov/pubmed/21266384. Accessed 1/1/2013.