Sertoli cell-only syndrome (SCOS), also known as germ cell aplasia, is defined by azoospermia where the testicular seminiferous tubules are lined solely with sertoli cells.[2] Sertoli cells contribute to the formation of the blood-testis barrier and aid in sperm generation. These cells respond to follicle-stimulating hormone, which is secreted by the hypothalamus and aids in spermatogenesis.[4]
Quick Facts Other names, Specialty ...
Sertoli cell-only syndrome |
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Other names | Germinal cell aplasia, Del Castillo syndrome, Germ cell aplasia. |
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Seminiferous tubules demonstrating maturation arrest. |
Specialty | Endocrinology, andrology |
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Symptoms | Infertility, azoospermia, testicular atrophy, high FSH levels.[1] |
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Causes | Y-chromosome microdeletions, chemical or toxin exposure, radiation therapy, or severe testicular injuries.[2] |
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Diagnostic method | Testicular biopsy.[3] |
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Differential diagnosis | Leydig cell hyperplasia, azoospermia, klinefelter syndrome, end-stage testis failure, maturation arrest, and hypospermatogenesis.[4] |
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Treatment | Microscopic testicular sperm extraction or testicular sperm aspiration.[1] |
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Frequency | Rare.[4] |
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Men often learn they have Sertoli cell-only syndrome between the ages of 20 and 40 when they are checked for infertility and found to produce no sperm. Other signs and symptoms are uncommon, yet in some cases, an underlying cause of SCO syndrome, such as Klinefelter syndrome, may produce other symptoms.[3]
Most cases of SCO syndrome are idiopathic, however, causes may include deletions of genetic material on Y-chromosome regions, particularly the azoospermia factor area. Other factors include chemical or toxin exposure, previous exposure to radiation therapy, and a history of severe trauma. A testicular biopsy confirms the diagnosis of SCO syndrome. Although there is no effective treatment at the moment, assisted reproductive technology may help some men with SCO syndrome reproduce.[3]
Infertility is the most prevalent symptom of Sertoli-cell-only syndrome. Semen examination reveals azoospermia, with sperm density frequently falling to fewer than 1 million sperm per mL. When sperm density falls, the testes exhibit SCO syndrome and hypospermatogenesis. The testes in men with SCO syndrome are normally small to normal in size, with normal form and consistency; though, some patients may have significant atrophy of the testes.[4] The majority of patients with Sertoli cell-only syndrome (up to 90%) have increased FSH levels, which are typically two to three times normal.[1]
Sertoli cell-only syndrome is usually initially assessed by conducting two separate semen analyses. Sertoli cell-only disease is frequently characterized by azoospermia which is the complete absence of sperm in semen. A tiny fraction of patients may still have measurable sperm levels.[1]
About 90% of those with Sertoli cell-only syndrome have elevated FSH levels, usually two to three times the normal amount.[1]
Testicular biopsy is the only way to confirm non-obstructive azoospermia and Sertoli cell-only syndrome.[1]
Sertoli cell-only syndrome currently has no known cure or treatment. However, patients who have significantly low sperm counts and even no sperm in the sperm may still be evaluated for assisted reproductive techniques. Microscopic testicular sperm extraction is a microsurgical procedure that extracts sperm straight from the patient's testes. It has a substantially greater sperm retrieval success rate than testicular sperm aspiration, which is performed using a simple needle stick but does not require a microscopic surgeon.[1]
Sertoli cell-only syndrome is extremely rare in the general population. Infertility affects around 10% of US couples. Approximately 30% of these couples have a pure male factor as the underlying cause, whereas the remaining 20% have a male and female influence. Although specific data are difficult to get, SCO syndrome affects less than 5%-10% of the aforementioned infertile individuals.[4]