TART in congenital adrenal hyperplasia: current insights

1 April 2019

Testicular adrenal rest tumors (TARTs) mostly occur in patients with congenital adrenal hyperplasie (CAH) one of the most common congenital adrenal diseases.

Hedi Claahsen and colleagues, theme Vascular damage, provide in Endocrine Reviews current insights on testicular adrenal rest tumors (TARTs),

In recent studies, an overall TART prevalence of 40% (range 14-89%) in classic CAH-patients is found. Reported differences are mainly caused by the method of detection, and the selected patient population. Biochemically, histologically, and molecularly TARTs exhibit particular adrenal characteristics and were therefore thought to originate from aberrant adrenal cells. More recently TART has been found to also exhibit testicular characteristics. This has led to the hypothesis of pluripotent cells as the origin of TART. High concentrations of adrenocorticotropic hormone (ACTH) could cause hyperplasia of these pluripotent cells, as TART appears to be associated with poor hormonal control with concomitant elevated ACTH. Unfortunately, as yet there are no methods to prevent the development of TART, nor are there guidelines to treat patients with TART. Intensified glucocorticoid treatment could improve fertility status in some cases, although studies report contradicting results. TART can also lead to irreversible testicular damage, therefore semen cryopreservation could be offered to patients with TART.

Further research should focus on the etiology and pharmacological treatment to prevent TART development or to treat TART and improve fertility status of patients with TART.