There is no debate that gonadal tissue having any Y chromosome component in phenotypic females requires removal as soon as the diagnosis is made to avoid the risk of malignant gonadal tumors. There is one exception to this rule. Because gonadal tumors occur relatively late in patients with complete androgen insensitivity, surgery is delayed until after puberty. An accomplished laparoscopist can attempt this procedure, with the option of laparotomy if the gonads prove to be inaccessible. Streak gonads have been removed in this fashion, as well as the testes in androgen insensitivity. With androgen insensitivity, the gonads can be close to the external iliac artery and herniated into the inguinal canals. The procedure is any more difficult, and care must be taken to extract the gonad from the inguinal canal to secure complete excision. It may also be necessary to make a little abdominal incision or a culdotomy to extract the gonad in order to avoid morcellation.
The uterus and tubes should be preserved for the possibility of pregnancy with donor oocytes.