Testicular feminization syndrome in three sibs: emphasis on gonadal neoplasia

Abnormal infantile germ cells and development of carcinoma‐in‐situ in maldeveloped testes: a stereological and densitometric study

“…Adults with AIS show predisposition to germ cell tumours (Dewhurst, 1970;Dewhurst et al, 1971;Manuel et al, 1976;Morris & Mahesh, 1963;O'Connell et al, 1973;Scully, 1981), and CIS of the adult type has been described in these patients (Nogales et al, 1981;Skakkebak, 1979). We have investigated 12 consecutive children and adolescents with a median age of 6 years (range 2 months-19 years) for the presence of CIS (Muller & Skakkebak, 1984); Muller, 1984).…”

Incidence of intratubular germ cell neoplasia in androgen insensitivity syndrome

“…These findings would seem to indicate that a rethink is needed concerning the general opinion that patients with androgen intensivity syndrome have practically no risk of developing malignancy, and that orchidectomy is not advisable before puberty is completed. It has long been recognized that patients with an¬ drogen insensitivity syndrome have a higher inci¬ dence of germ cell tumours (1,2) in adulthood.…”

Complete androgen insensitivity syndrome: Review of four cases

“…The reported incidences range from 1 in 20 000 to 1 in 62 400 live births [2,8]. Despite the reported literature data of familial inheritance [9], patient histories in our four cases were not significant.…”

“…Others consider the expectancy of the tumor to be a sufficient reason for early removal of both gonads [9]. There is still some controversy in prophylactic gonadectomy, so we reached the decision in each case through the consultation with the multidisciplinary expert team.…”