eMedicine Specialties > Urology > Male Infertility

Sertoli-Cell-Only Syndrome: Differential Diagnoses & Workup

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, Chief 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: Aug 14, 2009

Differential Diagnoses

Other Problems to Be Considered

Leydig cell hyperplasia
Azoospermia
Klinefelter syndrome
End-stage testis failure
Hypospermatogenesis
Maturation arrest

Workup

Laboratory Studies

  • Men undergoing evaluation for infertility typically undergo hormonal evaluation with follicle-stimulating hormone (FSH) and testosterone studies. Luteinizing hormone (LH) and prolactin testing are not routinely necessary. In addition, routine testing for male infertility includes several semen analyses.
    • Plasma testosterone levels are typically normal.
    • Azoospermia and an elevated serum FSH level (>2-3 times reference range) indicate spermatogenic failure.
    • Azoospermia and a serum FSH level within the reference range suggest possible spermatogenic failure or an obstruction.
    • The serum FSH level is typically (90%) elevated. Elevations of greater than 2.5-3 times the reference range are diagnostic for spermatogenic failure.
  • Findings on testicular biopsy may include severe hypospermatogenesis, maturation arrest-spermatid stage, maturation arrest-spermatocyte stage, or SCO syndrome.
  • If a couple is considering intracytoplasmic sperm injection (ICSI), a micromanipulation technique in which a single sperm is injected into an oocyte, they should be offered genetic testing with a Y-chromosome microdeletion assay and karyotyping.

Imaging Studies

  • Imaging studies are not indicated in SCO syndrome.

Other Tests

  • Azoospermia should be documented with a semen pellet analysis. The semen pellet is performed by centrifuging a grossly azoospermic semen specimen for 10 minutes at 1500-2000 rpm. This pellet is considered standard for the diagnosis of azoospermia. The pellet at the bottom of the conical tube is examined under a microscope at a magnification of 400X. If sperm are identified, then patchy spermatogenesis within the testes is present.
  • Men with SCO syndrome who are azoospermic are at an increased risk of testicular nodules and cancer. As such, the roles of clinical evaluation, ultrasonography, and biopsy should be emphasized.4

Procedures

  • The testis biopsy is the criterion standard for diagnosis of SCO syndrome. Most urologists are familiar with the open biopsy technique.
    • This technique may be performed with a local cord block in most men.
    • Alternatively, a general anesthetic may be used.
    • A small biopsy sample is taken from the surface of the testis and placed in Bouin fixative.
  • Men may be offered a testis biopsy to define whether an obstruction or spermatogenic failure is present. If the serum FSH level is greater than 2.5-3 times the reference range and intracytoplasmic sperm injection (ICSI) is not a consideration, the biopsy findings would not change the management plan. If the couple is considering ICSI, a diagnostic biopsy may be helpful to provide counseling regarding the possibility of finding sperm at the time of ICSI. If spermatogenic failure is predominant and rare sperm are identified, testicular extraction of sperm with ICSI may be possible at a later time. At the time of testis biopsy, a specimen may be cryopreserved for potential use of any sperm at a later time.
  • Some investigators discuss the use of fine-needle aspiration cytology (FNAC) prior to biopsy as a less invasive means of establishing a diagnosis. In addition, this technique can offer prognostic information regarding the likelihood of successful testicular sperm extraction (TESE).

Histologic Findings

Germinal cell aplasia (SCO syndrome) is histologically characterized by seminiferous tubules that contain only Sertoli cells, with a complete absence of all germ cells. In most cases, the tunica propria and basement membranes are not thickened appreciably, and the tubules are normal or slightly decreased in diameter. The interstitium contains normal numbers of Leydig cells. One may observe a patient with an otherwise classic example of germinal cell aplasia who has an occasional tubule with some degree of spermatogenesis. Levin has classified this as germinal cell aplasia and focal spermatogenesis.

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. Yang Y, Ma MY, Xiao CY, Li L, Li SW, Zhang SZ. Massive deletion in AZFb/b+c and azoospermia with Sertoli cell only and/or maturation arrest. Int J Androl. Dec 2008;31(6):573-8. [Medline].

  2. Ferlin A, Arredi B, Speltra E, Cazzadore C, Selice R, Garolla A, et al. Molecular and clinical characterization of Y chromosome microdeletions in infertile men: a 10-year experience in Italy. J Clin Endocrinol Metab. Mar 2007;92(3):762-70. [Medline].

  3. Kim SK, Yoon YD, Park YS, Seo JT, Kim JH. Involvement of the Fas-Fas ligand system and active caspase-3 in abnormal apoptosis in human testes with maturation arrest and Sertoli cell-only syndrome. Fertil Steril. Mar 2007;87(3):547-53. [Medline].

  4. Mancini M, Carmignani L, Gazzano G, Sagone P, Gadda F, Bosari S, et al. High prevalence of testicular cancer in azoospermic men without spermatogenesis. Hum Reprod. Apr 2007;22(4):1042-6. [Medline].

  5. 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].

  6. 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].

  7. Bettella A, Ferlin A, Menegazzo M, Ferigo M, Tavolini IM, Bassi PF, et al. Testicular fine needle aspiration as a diagnostic tool in non-obstructive azoospermia. Asian J Androl. Sep 2005;7(3):289-94. [Medline].

  8. Colpi GM, Piediferro G, Nerva F, Giacchetta D, Colpi EM, Piatti E. Sperm retrieval for intra-cytoplasmic sperm injection in non-obstructive azoospermia. Minerva Urol Nefrol. Jun 2005;57(2):99-107. [Medline].

  9. 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].

  10. Hibi H, Ohori T, Yamada Y, Honda N, Hashiba Y, Asada Y. Testicular sperm extraction and ICSI in patients with post-chemotherapy non-obstructive azoospermia. Arch Androl. Mar-Apr 2007;53(2):63-5. [Medline].

  11. 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].

  12. 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].

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

  14. 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].

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

  16. 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].

  17. Weller O, Yogev L, Yavetz H, Paz G, Kleiman S, Hauser R. Differentiating between primary and secondary Sertoli-cell-only syndrome by histologic and hormonal parameters. Fertil Steril. Jun 2005;83(6):1856-8. [Medline].

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Further Reading

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Keywords

Sertoli-cell-only syndrome, germinal cell aplasia, germ cell aplasia, SCO syndrome, 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; Astellas Consulting fee Speaking and teaching; Indevus Consulting fee Speaking and teaching

Coauthor(s)

Joe D Mobley III, MD, MPH, Chief 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, Professor, Department of Urology, College of Physicians and Surgeons, Columbia University; Director of Urology, Allen Pavilion, New York Presbyterian Hospital
Erik T Goluboff, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, 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: eMedicine Salary Employment

CME Editor

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

Chief Editor

Bradley Fields Schwartz, DO, FACS, Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine
Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, and Society of University Urologists
Disclosure: Nothing to disclose.

 
 
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