Complete androgen insensitivity syndrome and risk of gonadal malignancy: systematic review.

Autor: Barros BA; Interdisciplinary Group for the Study of Sex Determination and Differentiation (GIEDDS), State University of Campinas (UNICAMP), Campinas/São Paulo, Brazil.; Department of Pediatrics, State University of Campinas (UNICAMP), Campinas/São Paulo, Brazil., Oliveira LR; Department of Pediatrics, Federal University of Uberlândia (UFU), Uberlândia/Minas Gerais, Brazil., Surur CRC; Department of Pediatrics, State University of Campinas (UNICAMP), Campinas/São Paulo, Brazil., Barros-Filho AA; Department of Pediatrics, State University of Campinas (UNICAMP), Campinas/São Paulo, Brazil., Maciel-Guerra AT; Interdisciplinary Group for the Study of Sex Determination and Differentiation (GIEDDS), State University of Campinas (UNICAMP), Campinas/São Paulo, Brazil.; Department of Medical Genetics, State University of Campinas (UNICAMP), Campinas/São Paulo, Brazil., Guerra-Junior G; Interdisciplinary Group for the Study of Sex Determination and Differentiation (GIEDDS), State University of Campinas (UNICAMP), Campinas/São Paulo, Brazil.; Department of Pediatrics, State University of Campinas (UNICAMP), Campinas/São Paulo, Brazil.
Jazyk: angličtina
Zdroj: Annals of pediatric endocrinology & metabolism [Ann Pediatr Endocrinol Metab] 2021 Mar; Vol. 26 (1), pp. 19-23. Date of Electronic Publication: 2021 Mar 31.
DOI: 10.6065/apem.2040170.085
Abstrakt: Complete androgen insensitivity syndrome (CAIS) is a rare condition characterized by 46,XY karyotype, female external genitalia, absence of uterus, and testes located intra-abdominally, in the inguinal ring or in the labia majora. In the present study, the frequency of testicular malignancy in prepubertal and pubertal patients with CAIS who underwent gonadectomy or gonadal biopsy were evaluated. Systematic review was performed using electronic databases according to the PRISMA-P (Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols) guidelines. The samples included 15 articles published between 1998 and 2019. From a total of 456 patients who underwent gonadectomy or gonadal biopsy, 6.14% had a premalignant lesion and most were postpubertal (82.14%). A malignant lesion was found in 1.3% and all were postpubertal. Because the risk of malignancy is very low in prepubertal patients with CAIS, gonadectomy may be delayed until puberty is complete, allowing it to progress naturally; however, close follow-up of the patient is required.
Databáze: MEDLINE