Testis sparing surgery for steroid unresponsive testicular tumors of the congenital adrenal hyperplasia

Tiryaki, Tuğrul; Aycan, Zehra; Hücümenoğlu, Sema; Atayurt, Halil

doi:10.1007/s00383-005-1547-x

Cited by 39 publications

(41 citation statements)

References 10 publications

(13 reference statements)

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“…As a last resort, testis-sparing surgery might be considered in order to improve fertility. The results of surgery on fertility have been highly variable [174][175][176] and, in one of these studies, the recommendation was that surgery is only indicated for relief of pain and discomfort caused by the TARTs [176]. Surgery should probably only be considered for long-standing TARTs when testicular biopsies have demonstrated viable testicular tissue since surgery to improve fertility is uncertain.…”

Section: Testicular Adrenal Rest Tumors Tartsmentioning

confidence: 96%

Nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency: clinical presentation, diagnosis, treatment, and outcome

2015

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Nonclassic congenital adrenal hyperplasia (NCAH) is one of the most frequent autosomal recessive disorders in man with a prevalence ranging from 0.1 % in Caucasians up to a few percent in certain ethnic groups. Most cases are never diagnosed due to very mild symptoms, misdiagnosing as polycystic ovary syndrome, or ignorance. In contrast to classic CAH, patients with NCAH present with mild partial cortisol insufficiency and hyperandrogenism and will survive without any treatment. Undiagnosed NCAH may result in infertility, miscarriages, oligomenorrhea, hirsutism, acne, premature pubarche, testicular adrenal rest tumors, adrenal tumors, and voice problems among other symptoms. A baseline measurement of 17-hydroxyprogesterone can be used for diagnosis, but the ACTH stimulation test with measurement of 17-hydroxyprogesterone is regarded as the golden standard. The diagnosis can be verified by CYP21A2 mutation analysis. Treatment is symptomatic and usually with glucocorticoids alone. The lowest possible glucocorticoid dose should be used. Long-term treatment with glucocorticoids will improve the symptoms but will also result in iatrogenic cortisol insufficiency and may also lead to long-term complications such as obesity, insulin resistance, hypertension, osteoporosis, and fractures. Although the complications seen in NCAH patients have been assumed to be related to the glucocorticoid treatment, some may, in fact, be associated with prolonged hyperandrogenism. Different risk factors and negative consequences should be monitored regularly in an attempt to improve the clinical outcome. More research is needed in this relatively common disorder.

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Section: Testicular Adrenal Rest Tumors Tartsmentioning

confidence: 96%

Nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency: clinical presentation, diagnosis, treatment, and outcome

2015

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“…If the tumor size does not decrease, surgical intervention may be considered. Two studies have described testis-sparing enucleation of the tumor (21,22). However, whether improvement of gonadal function occurs after surgery remains unclear, so surgery should be reserved for the patients who do not respond to conservative treatment.…”

Section: Tablementioning

confidence: 96%

Repeated successful induction of fertility after replacing hydrocortisone with dexamethasone in a patient with congenital adrenal hyperplasia and testicular adrenal rest tumors

Grinten¹,

Otten²,

Sweep³

et al. 2007

Fertility and Sterility

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“…In cases of 21OHD with minimal response to increased dose of glucocorticoids or with persistent azoospermia, testis sparing surgery has been shown to improve fertility [150,151]. However, this is not a consistent finding and hence surgery should be performed only if testicular biopsy confirms viable testicular tissue [2,136].…”

Section: Testicular Adrenal Rest Tumorsmentioning

confidence: 98%

Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency

2016

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Congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency is a rare autosomal recessive genetic disorder. It is caused by reduced or absent activity of 11β-hydroxylase (CYP11B1) enzyme and the resultant defects in adrenal steroidogenesis. The most common clinical features of 11 beta-hydroxylase deficiency are ambiguous genitalia, accelerated skeletal maturation and resultant short stature, peripheral precocious puberty and hyporeninemic hypokalemic hypertension. The biochemical diagnosis is based on raised serum 11-deoxycortisol and 11-deoxycorticosterone levels together with increased adrenal androgens. More than 100 mutations in CYP11B1 gene have been reported to date. The level of in-vivo activity of CYP11B1 relates to the degree of severity of 11 beta-hydroxylase deficiency. Clinical management of 11 beta-hydroxylase deficiency can pose a challenge to maintain adequate glucocorticoid dosing to suppress adrenal androgen excess while avoiding glucocorticoid-induced side effects. The long-term outcomes of clinical and surgical management are not well studied. This review article aims to collate the current available data about 11 beta-hydroxylase deficiency and its management.

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Testis sparing surgery for steroid unresponsive testicular tumors of the congenital adrenal hyperplasia

Cited by 39 publications

References 10 publications

Nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency: clinical presentation, diagnosis, treatment, and outcome

Nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency: clinical presentation, diagnosis, treatment, and outcome

Repeated successful induction of fertility after replacing hydrocortisone with dexamethasone in a patient with congenital adrenal hyperplasia and testicular adrenal rest tumors

Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency

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