[Bilateral testicular tumor in a young man with congenital 11β-hydroxylase deficiency].
Adrenal rest tumor presenting as palpable testicular mass has been well described in boys and adult males with congenital adrenal hyperplasia. It develops most commonly in patients with 21- hydroxylase deficiency, but the entity may also occur in rare forms of congenital adrenal hyperplasia, including 11β-hydroxylase deficiency. Because the management of testicular adrenal rest tumors is substantially different from that applied in benign and malignant testicular tumors, an accurate differentiation between these entities is particularly important. Authors present the history of a young adult male with 11β-hydroxylase deficiency who developed adrenal rest tumors presenting as palpable bilateral testicular masses during treatment with glucocorticoids, then testicular masses showed a rapid regression after an adequate glucocorticoid treatment. Considering lessons obtained from this case, authors review the pathomechanism, symptoms, as well as current diagnostic and treatment modalities of testicular adrenal rest tumors.
Vajda
Zsolt
Z
Fovárosi Önkormányzat Heim Pál Kórház Budapest Üllői út 86. 1089. zsvajda@heimpalkorhaz.hu
Borbély
Gergo
G
Jakab
Zsuzsa
Z
Riesz
Péter
P
Rácz
Károly
K
D000314:Adrenal Rest Tumor / Q000175:diagnosis / Q000188:drug therapy / Q000201:enzymology; D000328:Adult; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D013252:Steroid 11-beta-Hydroxylase / Q000378:metabolism*; D013736:Testicular Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000201:enzymology
D005938:Glucocorticoids; D013252:Steroid 11-beta-Hydroxylase
11-β-hidroxiláz enzim defektusában szenvedő fiatal férfi kétoldali heredaganata.