Persistent Müllerian Duct Syndrome

Case report: crossed testicular ectopia

“…Laparoscopic-assisted transabdominal orchiopexy has been described and may offer alternative to orchiectomy in cases lacking adequate spermatic cord length [8]. Orchiectomy should be reserved for cases where ectopic testes cannot be mobilized to a palpable position given the future risk of malignancy [9]. Addition of pelvic ultrasound to the preoperative evaluation of patients with inguinal hernia and contralateral cryptorchidism may detect ectopic testes and associated congenital abnormalities allowing for more definitive operative planning.…”

Uterine adenosarcoma in a boy with persistent müllerian duct syndrome: first reported case

“…Persistent mqllerian duct syndrome is a rare autosomal recessive form of male pseudohermaphroditism first described by Nilson in 1939 as hernia uteri inguinale [1,2]. Bilateral fallopian tubes and a uterus occur with an otherwise normal male genotype (46,XY) and normal external male genitalia.…”

“…Leaving the uterus and fallopian tubes in situ is indicated because malignant degeneration rarely occurs [1]. It is feared that removal of the remnants may compromise fertility secondary to traumatic damage to the ductus deferens and testis [1].…”

“…D 2005 Elsevier Inc. All rights reserved. Persistent mqllerian duct syndrome (PMDS) is a rare autosomal recessive form of male pseudohermaphroditism [1,2]. Testicular neoplasm in PMDS has been reported, but malignant degeneration of the remnant female genitalia has only once been reported [3].…”

Persistent Müllerian Duct Syndrome with Seminoma

“…Ideally it is complemented by karyotyping [8]. Imaging features, although rarely encountered, are characteristic.…”