Prevalence of Testicular Adrenal Rest Tumor and Factors Associated with Its Development in Congenital Adrenal Hyperplasia

Autor(es)

Mendes-dos-Santos, Carolina Taddeo ^[1] ; Martins, Daniel Lahan ^[2] ; Guerra-Junior, Gil ^[1] ; Matias Baptista, Maria Tereza ^[1] ; de-Mello, Maricilda Palandi ^[3] ; de Oliveira, Laurione Candido ^[4] ; Morcillo, Andre Moreno ^[1] ; Valente Lemos-Marini, Sofia Helena ^[1]

Afiliação do(s) autor(es)

^[1] Univ Estadual Campinas, UNICAMP, Dept Pediat, Pediat Endocrinol Unit, CIPED, Campinas, SP - Brazil

^[2] Univ Estadual Campinas, UNICAMP, Dept Radiol, Campinas, SP - Brazil

^[3] Univ Estadual Campinas, UNICAMP, Ctr Mol Biol & Genet Engn CBMEG, Campinas, SP - Brazil

^[4] Univ Estadual Campinas, UNICAMP, Clin Hosp, Lab Physiol, Campinas, SP - Brazil

• Tipo de documento: Artigo Científico
• Fonte: Hormone Research in Paediatrics; v. 90, n. 3, p. 161-168, 2018.
• Citações Web of Science: 3

Resumo

Background: Testicular adrenal rest tumors (TART) can cause infertility in congenital adrenal hyperplasia (CAH) males. Aims: To determine TART prevalence in patients with CAH due to 21-hydroxylase deficiency (21-OHD) and evaluate possible factors associated with its development. Methods: This is a descriptive and analytical cross-sectional study evaluating males with the classical form of 21-OHD through testicular ultrasonography and serum inhibin B dosages. Data on prescribed glucocorticoid dose and serum levels of 17 hydroxyprogesterone (17-OHP), androstenedione (Andro), ACTH, renin, and LH were obtained from medical records. Results: Thirty-eight males were evaluated. The mean age on ultrasonography was 15.2 +/- 6.7 (3-27) years. Nine patients (23.7%) had TART, 4 of them were prepubertal and the youngest was 5 years old. No association was found between TART and 21-OHD phenotype, glucocorticoid dose, or 17OHP, ACTH, LH, renin, and inhibin B levels measured in the 6 preceding years. However, 50% of the patients who presented increased Andro 2 years prior to the evaluation had TART (p = 0.018, OR = 8.00 {[}95% CI: 1.42-44.92]), whereas in the normal Andro group only 16.7% had tumors. Conclusion: This study showed that TART can occur in prepubertal patients and that disease control could be a factor associated with its development. Therefore, we suggest investigating TART development early in childhood, mainly in poorly controlled 21-OHD patients. (c) 2018 S. Karger AG, Basel (AU)

• Processo FAPESP: 11/51808-2 - Estudo da expressao do gene cyp21a2 e da atividade enzimatica da 21-hidroxilase resultante de mutacoes raras em casos de hyperplasia da adrenal congenita nas formas classica e tardia.
• Beneficiário: Maricilda Palandi de Mello
• Modalidade de apoio: Auxílio à Pesquisa - Regular