Approach Considerations

No effective medical therapy exists for Sertoli-cell-only (SCO) syndrome. If the couple is considering in vitro fertilization/intracytoplasmic sperm injection (IVF/ICSI), consultation with a reproductive endocrinologist is necessary.

Surgical Care

Testicular sperm extraction (TESE) may be offered to couples considering IVF/ICSI. At specialty centers, as many as 20%-40% of men with SCO syndrome may be identified as having isolated foci of spermatogenesis within the testis; however, the option of using donor sperm must be discussed with the couple. At most centers, sperm recovery rates are much lower.

TESE is a testis biopsy performed with the intent of finding mature sperm within the seminiferous tubules. Multiple and extensive biopsies are typically required when SCO syndrome is present. Because spermatogenesis may be patchy within the testis, occasional pockets of isolated sperm production may be identified, even when the predominant histopathology finding is SCO syndrome.

Microsurgical TESE (mTESE) may be performed at some specialty centers, offering improved chances of successful sperm extraction with a decreased risk of morbidity.

In patients with SCO syndrome with pockets of sperm production, repair of a concurrent varicocele can increase the chances of subsequent successful surgical retrieval of sperm.

A meta-analysis of the outcomes of 37 trials enrolling a total of 1248 men with Klinefelter syndrome who underwent TESE found that sperm retrieval rates per TESE cycle of ranged from 39-48%. Similar results were observed for in both conventional TESE (35-50%) and mTESE (38-52%). Overall a total of 218 pregnancies occurred after 410 ICSI cycles for a pregnancy rate of 43%. Meta-regression analysis showed that age; testis volum;e and FSH, LH, and testosterone levels at enrollment had no effect on the final sperm retrieval or pregnancy rate.^[17]

Medication

Ramphul K, Mejias SG. Sertoli-Cell-Only Syndrome. 2021 Jan. [QxMD MEDLINE Link]. [Full Text].
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. 2003 Jun. 125(6):769-84. [QxMD MEDLINE Link].
Lei B, Wan B, Peng J, Yang Y, Lv D, Zhou X, et al. PRPS2 Expression Correlates with Sertoli-Cell Only Syndrome and Inhibits the Apoptosis of TM4 Sertoli Cells. J Urol. 2015 Nov. 194 (5):1491-7. [QxMD MEDLINE Link].
Taniguchi H, Katano T, Nishida K, Yao I, Morimoto Y, Matsuda T, et al. Expression of hOvol2 in the XY body of human spermatocytes. Andrologia. 2017 Feb. 49 (1):[QxMD MEDLINE Link]. [Full Text].
Yao C, Sun M, Yuan Q, Niu M, Chen Z, Hou J, et al. MiRNA-133b promotes the proliferation of human Sertoli cells through targeting GLI3. Oncotarget. 2016 Jan 19. 7 (3):2201-19. [QxMD MEDLINE Link]. [Full Text].
Miyamoto T, Koh E, Tsujimura A, Miyagawa Y, Saijo Y, Namiki M, et al. Single-nucleotide polymorphisms in the LRWD1 gene may be a genetic risk factor for Japanese patients with Sertoli cell-only syndrome. Andrologia. 2013 Feb 28. [QxMD MEDLINE Link].
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. 2008 Dec. 31(6):573-8. [QxMD MEDLINE Link].
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. 2007 Mar. 92(3):762-70. [QxMD MEDLINE Link].
Hadjkacem-Loukil L, Hadj-Kacem H, Hadj Salem I, Bahloul A, Fakhfakh F, Ayadi H. Genotyping of Tunisian azoospermic men with Sertoli cell-only and maturation arrest. Andrologia. 2011 Jul 6. [QxMD MEDLINE Link].
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. 2007 Mar. 87(3):547-53. [QxMD MEDLINE Link].
Stouffs K, Gheldof A, Tournaye H, Vandermaelen D, Bonduelle M, Lissens W, et al. Sertoli Cell-Only Syndrome: Behind the Genetic Scenes. Biomed Res Int. 2016. 2016:6191307. [QxMD MEDLINE Link]. [Full Text].
Kavoussi PK, Hunn C, Gilkey MS, Chen SH, Kavoussi KM, Wininger JD, et al. Sertoli cell only syndrome induced by a varicocele. Transl Androl Urol. 2019 Aug. 8 (4):405-408. [QxMD MEDLINE Link]. [Full Text].
Sangwan JS, Petit C, Rose RS, Frapsauce C, Dijols L, Rigot JM, et al. Non-obstructive idiopathic azoospermia vs azoospermia with antecedents of cryptorchidism: ways and probabilities of becoming parents. Basic Clin Androl. 2021 Dec 9. 31 (1):30. [QxMD MEDLINE Link]. [Full Text].
Cissen M, Meijerink AM, D'Hauwers KW, Meissner A, van der Weide N, Mochtar MH, et al. Prediction model for obtaining spermatozoa with testicular sperm extraction in men with non-obstructive azoospermia. Hum Reprod. 2016 Sep. 31 (9):1934-41. [QxMD MEDLINE Link]. [Full Text].
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. 2007 Apr. 22(4):1042-6. [QxMD MEDLINE Link].
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. 2005 Sep. 7(3):289-94. [QxMD MEDLINE Link].
Corona G, Pizzocaro A, Lanfranco F, Garolla A, Pelliccione F, Vignozzi L, et al. Sperm recovery and ICSI outcomes in Klinefelter syndrome: a systematic review and meta-analysis. Hum Reprod Update. 2017 May 1. 23 (3):265-275. [QxMD MEDLINE Link].

Media Gallery

This hematoxylin and eosin section of a testis biopsy (400X) demonstrates an individual tubule lined only with Sertoli cells (Sertoli-cell-only [SCO] syndrome). The Sertoli cells line the seminiferous tubule.
Interaction between the hypothalamus and the testes. Courtesy of Wikispaces at https://malereprobio12.wikispaces.com/.

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Author

Edward David Kim, MD, FACS Professor of Urology, Department 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 Society for Reproductive Medicine, American Urological Association, Sexual Medicine Society of North America, Society for Male Reproduction and Urology, Society for the Study of Male Reproduction, Tennessee Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Joe D Mobley, III, MD, MPH Urologist, Kentucky Lake Urology Clinic

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, Tennessee Medical Association

Disclosure: Nothing to disclose.

Adam F Stewart, MD Resident Physician, Department of Surgery, Division of Urology, University of Tennessee Medical Center, University of Tennessee Graduate School of Medicine

Disclosure: Nothing to disclose.

Jared Moss, MD Resident Physician, Division of Urology, University of Tennessee Graduate School of Medicine

Jared Moss, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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 Laparoscopic and Robotic Surgeons, Society of University Urologists

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Endourological Society Board of Directors; President Elect North Central Section of the American Urological Association<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cook Medical.

Additional Contributors

Erik T Goluboff, MD Professor, Department of Urology, College of Physicians and Surgeons, Columbia University College of Physicians and Surgeons; 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, Society for Basic Urologic Research

Disclosure: Nothing to disclose.

Sertoli-Cell-Only Syndrome Treatment & Management

Approach Considerations

Surgical Care

References

Tables

Contributor Information and Disclosures

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