Effects of mitotane on testicular adrenal rest tumors in congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a retrospective series of five patients

in European Journal of Endocrinology
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  • 1 AP-HP, IE3M, Hôpital Pitié-Salpêtrière, Department of Endocrinology and Reproductive Medicine and Centre de Référence des Maladies Endocriniennes Rares de la Croissance et du Développement, Paris, France
  • 2 Sorbonne Université, Paris, France
  • 3 Department of Endocrinology, Hospices Civils de Lyon, Fédération d'Endocrinologie, Bron, France
  • 4 AP-HP, Hôpital Pitié-Salpêtrière, Department of Hormonal Biochemistry
  • 5 AP-HP, Hôpital Pitié-Salpêtrière, Department of Radiology, Paris, France

Correspondence should be addressed to A Bachelot; Email: anne.bachelot@aphp.fr
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We conducted a retrospective study on the long-term effect of mitotane treatment on testicular adrenal rest tumors (TARTs) in five adult patients with classic 21-hydroxylase deficiency. After 60 months of mitotane treatment, a decrease in adrenal steroids was observed in four patients. Testicular ultrasonography showed complete disappearance of TART in two patients, stabilization in two patients and a halving of TART volume in the remaining patient. Sperm count improved notably in two patients who had normal baseline inhibin B levels and small inclusions, thus enabling cryopreservation of the subjects’ semen. Four years of follow-up of these two patients after the withdrawal of mitotane showed no recurrence of TART and persistent normal testicular function. In conclusion, mitotane could be used as a last resort in CAH patients in the cases of azoospermia associated with TARTs but normal inhibin B levels, as it can improve long-term endocrine and exocrine testicular function.

 

     European Society of Endocrinology

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