hrp0084fc6.6 | Gonads & DSD | ESPE2015

Frequency of Gonadal Tumour in Complete Androgen Insensitivity Syndrome: a Retrospective Case-Series Analysis

Chaudhry Saduf , Tadokoro-Cuccaro Rieko , Hughes Ieuan , Acerini Carlo

Background: Complete androgen insensitivity syndrome (CAIS) is an X-linked recessive disorder of sex development (DSD) where affected individuals are phenotypically female but have a XY karyotype and testes. With increasing age there is an increased risk of malignant gonadal change, with incidence rates variously reported from 3.6 to 14%. Consequently, gonadectomy is recommended either during childhood or after puberty is complete, although there is no consensus on the optimal...