RESUMO
AIM: To analyse the urinary steroid metabolome in a boy who had true precocious puberty after a Leydig cell tumour. METHOD: Case report and detailed description of clinical and metabolic findings in a 7-year-old-boy with a Leydig cell tumour. RESULTS: Before surgery, the urinary steroid metabolome showed an activation of an alternative route to gonadal androgens independent of dehydroepiandrosterone (DHEA). After surgery, the boy entered true precocious puberty. Under leuprolide acetate treatment, clinical and laboratory findings normalized. CONCLUSION: Central precocious puberty after precocious pseudopuberty may be more common than expected and should be considered in children with persistent or recurrent symptoms after initial treatment of precocious pseudopuberty. Patients with a Leydig cell tumour seem to reactivate the so-called 'back door pathway' of androgen production, which is independent of the classical route via DHEA.
Assuntos
Leuprolida/uso terapêutico , Tumor de Células de Leydig/urina , Puberdade Precoce/tratamento farmacológico , Neoplasias Testiculares/urina , Androsterona/urina , Antineoplásicos Hormonais/uso terapêutico , Criança , Desidroepiandrosterona/urina , Etiocolanolona/urina , Humanos , Tumor de Células de Leydig/metabolismo , Tumor de Células de Leydig/cirurgia , Masculino , Metaboloma/fisiologia , Pregnanolona/urina , Puberdade Precoce/etiologia , Neoplasias Testiculares/metabolismo , Neoplasias Testiculares/cirurgia , Testosterona/urinaRESUMO
We report a case of unilateral Leydig cell tumor associated with gynecomastia and elevated urinary estrogens. Nine years after orchiectomy urinary estrogens became elevated and Leydig cell hyperplasia but no distinct tumor was identified in the remaining contralateral testicle.
Assuntos
Estrogênios/urina , Tumor de Células de Leydig/patologia , Células Intersticiais do Testículo/patologia , Neoplasias Testiculares/patologia , Adulto , Humanos , Hiperplasia , Tumor de Células de Leydig/complicações , Tumor de Células de Leydig/cirurgia , Tumor de Células de Leydig/urina , Masculino , Orquiectomia , Neoplasias Testiculares/complicações , Neoplasias Testiculares/cirurgia , Neoplasias Testiculares/urinaRESUMO
A 28-year-old man with evidence of feminization was demonstrated after 4 years of investigation to have a estrogen-secreting interstitial cell tumor. Such feminizing neoplasms are uncommon, only 37 having been described. They are usually benign and are characterized by gynecomastia, a testicular mass and, with lesser frequency, by decreased libido and potency and poor semen quality. The urinary excretion and plasma levels of estrogen are increased and, by selective testicular catheterization, the site of increased estrogen production can be localized. Secondary histologic changes occur in the nontumorous portions of the testis as well as in the contralateral testis; they are most marked in the area immediately adjacent to the tumor. Postoperatively, the gynecomastia regresses, the excessive levels of estrogen return to normal, libido improves, and the sperm count increases to normal.