Familial complete androgen insensitivity syndrome with prostatic tissue and seminal vesicles.
BACKGROUND
Complete androgen insensitivity syndrome (CAIS) is a rare androgen receptor function disorder where phenotypic female has a male genotype. They mostly present as bilateral inguinal hernias containing testes. Uterus and cervix are absent.


METHODS
The authors present a case of CAIS in a 22-year-old female where the presence of testes, prostatic tissue and seminal vesicles was confirmed by ultrasonography, hormonal analysis, operative findings and histopathological study. She was second of the two sisters and her elder sister was also diagnosed with CAIS.


CONCLUSIONS
High levels of testosterone seen in CAIS can stimulate Wolffian duct development/differentiation. Gonadectomy is advisable in such patients to avoid future neoplastic changes.
Tripathy
Kalpalata
K
Department of Pathology, S.C.B. Medical College, Cuttack 753007, Orissa, India. kalpalata_tripathy@yahoo.co.in
Gouda
Kalyaniprava
K
Palai
Pramod Kumar
PK
Das
Lucy
L
D013734:Androgen-Insensitivity Syndrome / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D010641:Phenotype; D011467:Prostate / Q000473:pathology* / Q000601:surgery; D011944:Receptors, Androgen / Q000235:genetics*; D013737:Testis / Q000473:pathology* / Q000601:surgery; D055815:Young Adult
C410798:AR protein, human; D011944:Receptors, Androgen