Testicular adrenal rest tumors (TART) in adult men with classic congenital adrenal hyperplasia (CAH)

• Published in: Urologe. Ausgabe A Vol. 47; no. 12; pp. 1596 - 1602
• Main Authors: Knape, P, Reisch, N, Dörr, H-G, Reincke, M, Lenk, S, Quinkler, M
• Format: Journal Article
• Language: German
• Published: Germany 01.12.2008
• Subjects: Adrenal Hyperplasia, Congenital - diagnosis; Adrenal Hyperplasia, Congenital - pathology; Adrenal Hyperplasia, Congenital - therapy; Adrenal Rest Tumor - diagnosis; Adrenal Rest Tumor - pathology; Adrenal Rest Tumor - therapy; Glucocorticoids - administration & dosage; Humans; Infertility, Male - etiology; Male; Mineralocorticoids - administration & dosage; Orchiectomy; Prognosis; Testicular Neoplasms - diagnosis; Testicular Neoplasms - pathology; Testicular Neoplasms - therapy; Testis - pathology
• ISSN: 0340-2592
• DOI: 10.1007/s00120-008-1857-4

Information about treatment of adult male patients with congenital adrenal hyperplasia (CAH) and testicular adrenal rest tumors (TART) is scarce. Diagnostic and therapeutic guidelines do not exist. The aim of this review is to evaluate the current state of therapeutic options in adult male patients with CAH. We performed an extensive search of the literature of the last 10 years by using PubMed/MEDLINE. The aims of treatment in adult male patients with CAH are prevention of adrenal crisis and TART, improvement of general well-being, good quality of life and sexual well-being, fertility, and prevention of side effects of gluco- and mineralocorticoid therapy. However, fertility is impaired in these patients and correlates with TART. The current therapeutic concepts are discussed. A future system of regular follow-up visits and standards in therapeutic concepts is needed to guarantee an improved fertility and lifelong good quality of life in adult male patients with CAH.