eMedicine Specialties > Urology > Male Infertility

Sertoli-Cell-Only Syndrome

Author: Edward David Kim, MD, FACS, Professor of Surgery, Division of Urology, University of Tennessee Graduate School of Medicine; Consulting Staff, University of Tennessee Medical Center
Coauthor(s): Joe D Mobley III, MD, MPH, Resident Physician, Department of Surgery, Division of Urology, University of Tennessee Graduate School of Medicine/University of Tennessee Medical Center
Contributor Information and Disclosures

Updated: Feb 1, 2007

Introduction

Background

Sertoli-cell-only (SCO) syndrome, also called germ cell aplasia, describes a condition of the testes in which only Sertoli cells line the seminiferous tubules. Typically, these men present between age 20-40 years for evaluation of infertility and are found to be azoospermic, a term describing the absence of sperm in the ejaculate. Physical examination is often unremarkable, and the diagnosis is made based on testicular biopsy findings. While investigation to identify a cause of SCO syndrome is ongoing, the etiology and mechanism of this process are currently unknown. No known effective treatment exists.

Pathophysiology

SCO syndrome is a condition of the testes. Involvement of other organ systems is rare but is secondary to the underlying condition causing SCO syndrome. As an example, Klinefelter syndrome is characterized by SCO and Leydig cell hyperplasia.

Frequency

United States

The prevalence of SCO syndrome in the overall population is extremely low. Approximately 10% of US couples are affected by infertility. Of these couples, approximately 30% have a pure male factor as the underlying cause, and another 20% have a combined male and female factor. Although precise figures are difficult to obtain, less than 5-10% of these infertile men have SCO syndrome.

Mortality/Morbidity

SCO syndrome presents during the evaluation of azoospermia in couples having difficulty in initiating a pregnancy. These men typically present with infertility as the only symptom.

Race

No known racial predilection for SCO exists; however, SCO presents more frequently in white men. In most series, most couples who present for evaluation of male infertility are white.

Sex

SCO syndrome affects only phenotypic men.

Age

The most common age of presentation is from 20-40 years. These age groups represent men who are trying to initiate a pregnancy.

Clinical

History

  • The most common presentation is a young man seeking evaluation for infertility.
    • His semen analysis has demonstrated azoospermia, the absence of sperm.
    • Azoospermia may be due to spermatogenic failure or obstruction. Examples of causes of spermatogenic failure include genetic, hormonal, idiopathic, toxin exposure, history of radiation therapy, and history of severe trauma. These conditions may be associated with Sertoli-cell-only (SCO) syndrome. Obstruction would not be associated with SCO.
    • Less commonly, these men may have severely decreased sperm densities of less than 1 million sperm per mL. In this latter situation, the testes have foci of SCO and hypospermatogenesis.
  • SCO is diagnosed with testicular biopsy.
    • Sperm production may be patchy and heterogenous within and between the testes.
    • In its purest sense, SCO must present as azoospermia; however, a minority of men may have foci of spermatogenesis in a testis that is predominantly SCO.

Physical

  • The testes are usually small to normal in size, with a normal shape and consistency.
  • The testes also may present with more marked atrophy.
  • Patients exhibit normal virilization without gynecomastia.
  • The remainder of the physical examination findings are typically unrevealing.

Causes

  • Most causes are idiopathic. A congenital absence of germ cells due to failure of migration of gonocytes is theoretically possible.
  • In the future, genetic causes likely will be identified. Y chromosome microdeletions are occasionally identified as a cause of SCO.
  • Exposure to chemicals and toxins may cause SCO; however, direct cause-and-effect relationships in humans have been difficult to document.
  • Klinefelter syndrome, 47 XXY, has a characteristic biopsy appearance of SCO and Leydig cell hyperplasia.
  • Attempting to distinguish between primary (congenital) and secondary (acquired) SCO syndrome is of no prognostic significance.

More on Sertoli-Cell-Only Syndrome

Overview: Sertoli-Cell-Only Syndrome
Differential Diagnoses & Workup: Sertoli-Cell-Only Syndrome
Treatment & Medication: Sertoli-Cell-Only Syndrome
Follow-up: Sertoli-Cell-Only Syndrome
Multimedia: Sertoli-Cell-Only Syndrome
References
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References

  1. Amer M, Haggar SE, Moustafa T. Testicular sperm extraction: impact of testicular histology on outcome, number of biopsies to be performed and optimal time for repetition. Hum Reprod. Dec 1999;14(12):3030-4. [Medline].

  2. Anniballo R, Ubaldi F, Cobellis L, et al. Criteria predicting the absence of spermatozoa in the Sertoli cell-only syndrome can be used to improve success rates of sperm retrieval. Hum Reprod. Nov 2000;15(11):2269-77. [Medline].

  3. Bettella A, Ferlin A, et al. Testicular fine needle aspiration as a diagnostic tool in non-obstructive azoospermia. Asian Journal of Andrology. 2005;7 (3):289-294.

  4. Colpi GM, Piediferro G, et al:. Sperm retrieval for intra-cytoplasmic sperm injection in non-obstructive azoospermia. Minerva Urologia e Nefrologia. 2005;57 (2):99-107.

  5. Ferras C, Fernandes S, Marques CJ, et al. AZF and DAZ gene copy-specific deletion analysis in maturation arrest and Sertoli cell-only syndrome. Mol Hum Reprod. Oct 2004;10(10):755-61. [Medline].

  6. Mancini M, Carmignani L, Gazzano G. High prevalence of testicular cancer in azoospermic men without spermatogenesis. Hum Reprod. Jan 12 2007.

  7. Nistal M, Jimenez F, Paniagua R. Sertoli cell types in the Sertoli-cell-only syndrome: relationships between Sertoli cell morphology and aetiology. Histopathology. Feb 1990;16(2):173-80. [Medline].

  8. Okada H, Dobashi M, Yamazaki T, et al. Conventional versus microdissection testicular sperm extraction for nonobstructive azoospermia. J Urol. Sep 2002;168(3):1063-7. [Medline].

  9. Schlegel PN, Palermo GD, Goldstein M. Testicular sperm extraction with intracytoplasmic sperm injection for nonobstructive azoospermia. Urology. Mar 1997;49(3):435-40. [Medline].

  10. Sharpe RM, McKinnell C, Kivlin C, Fisher JS. Proliferation and functional maturation of Sertoli cells, and their relevance to disorders of testis function in adulthood. Reproduction. Jun 2003;125(6):769-84. [Medline].

  11. Silber SJ. Sertoli cell only syndrome. Hum Reprod. Jan 1996;11(1):229. [Medline].

  12. Tournaye H, Liu J, Nagy PZ. Correlation between testicular histology and outcome after intracytoplasmic sperm injection using testicular spermatozoa. Hum Reprod. Jan 1996;11(1):127-32. [Medline].

  13. Weller O, Yogev L, et al. Differentiating between primary and secondary sertoli cell-only syndrome by histologic and hormonal parameters. Fertility and Sterility. Jun 2005;83 (6):1856-1858.

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Further Reading

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Keywords

Sertoli-cell-only syndrome, germinal cell aplasia, SCO, infertility, azoospermatism, azoospermic, Klinefelter syndrome, azoospermia, spermatogenic failure, spermatogenic obstruction

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Contributor Information and Disclosures

Author

Edward David Kim, MD, FACS, Professor of Surgery, Division of Urology, University of Tennessee Graduate School of Medicine; Consulting Staff, University of Tennessee Medical Center
Edward David Kim, MD, FACS is a member of the following medical societies: American College of Surgeons, American Society for Reproductive Medicine, American Society of Andrology, American Urological Association, and Tennessee Medical Association
Disclosure: Lilly Consulting fee Consulting

Coauthor(s)

Joe D Mobley III, MD, MPH, Resident Physician, Department of Surgery, Division of Urology, University of Tennessee Graduate School of Medicine/University of Tennessee Medical Center
Joe D Mobley III, MD, MPH is a member of the following medical societies: American College of Surgeons, American Medical Association, American Urological Association, Endourological Society, and Tennessee Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Erik T Goluboff, MD, Assistant Professor, Program Director, Department of Urology, Columbia-Presbyterian Medical Center, Columbia University
Erik T Goluboff, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Clinical Oncology, American Urological Association, Medical Society of the State of New York, New York Academy of Medicine, Phi Beta Kappa, and Society for Basic Urologic Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

CME Editor

J Stuart Wolf, Jr, MD, FACS, David A Bloom Professor of Urology, Director, Division of Minimally Invasive Urology, Department of Urology, University of Michigan Medical Center
J Stuart Wolf, Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Medical Association, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, and Society of University Urologists
Disclosure: Terumo Corporation Consulting fee Consulting; Omeros Corporation Consulting fee Consulting

Chief Editor

Stephen W Leslie, MD, FACS, Founder and Medical Director of the Lorain Kidney Stone Research Center, Clinical Assistant Professor, Department of Urology, Medical College of Ohio
Stephen W Leslie, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, National Kidney Foundation, and Ohio State Medical Association
Disclosure: Nothing to disclose.

 
 
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