Journal
ANNALS OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
Volume 26, Issue 1, Pages 19-23Publisher
KOREAN SOC PEDIATRIC ENDOCRINOLOGY
DOI: 10.6065/apem.2040170.085
Keywords
Androgen insensitivity syndrome; Gonadectomy; Neoplasia; Puberty
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The study found that the proportion of patients with premalignant lesions in CAIS patients was 6.14%, and the proportion of malignant lesions was 1.3%, with the majority occurring in postpubertal patients. Therefore, the risk of malignant lesions in prepubertal CAIS patients is very low, and gonadectomy can be delayed.
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.
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