Persistent Mullerian duct syndrome presenting with bilateral intra-abdominal gonadal tumours and obstructive uropathy.
Persistent Mullerian duct syndrome is a rare, autosomal recessive intersex disorder characterized by the presence of completely developed Mullerian duct derivatives in an otherwise normally virilized male with a 46, XY karyotype. We discuss a rare presentation of this disorder, bilateral gonadal tumours with obstructive uropathy, and its management, together with a review of the literature.
Asthana
S
S
Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi.
Deo
S V
SV
Shukla
N K
NK
Raina
V
V
Kumar
L
L
D000008:Abdominal Neoplasms / Q000150:complications / Q000175:diagnosis*; D000328:Adult; D003456:Cryptorchidism / Q000150:complications; D012734:Disorders of Sex Development / Q000150:complications* / Q000235:genetics / Q000473:pathology; D018237:Germinoma / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D006869:Hydronephrosis / Q000150:complications*; D007621:Karyotyping; D008297:Male; D009095:Mullerian Ducts; D013577:Syndrome; D013736:Testicular Neoplasms / Q000150:complications / Q000175:diagnosis*