Complete androgen insensitivity syndrome and risk of gonadal malignancy: systematic review
| Autor: | Beatriz Amstalden Barros, Letícia Ribeiro de Oliveira, Cíntia Regina Crocetti Surur, Antonio de Azevedo Barros-Filho, Andrea Trevas Maciel-Guerra, Gil Guerra-Junior |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: | |
| Zdroj: | Annals of Pediatric Endocrinology & Metabolism, Vol 26, Iss 1, Pp 19-23 (2021) |
| Druh dokumentu: | article |
| ISSN: | 2287-1012 2287-1292 19692501 |
| DOI: | 10.6065/apem.2040170.085 |
| Popis: | 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: | Directory of Open Access Journals |
| Externí odkaz: |
|