Congenital Abnormalities ; pathology ; Endometrial Neoplasms ; pathology ; Female ; Humans ; Kidney ; abnormalities ; pathology ; Kidney Diseases ; congenital ; pathology ; Middle Aged ; Mullerian Ducts ; abnormalities

OBJECTIVETo study the etiopathogenesis, clinical manifestations, diagnosis and management of persistent Müllerian duct syndrome (PMDS).

METHODSTwo cases of PMDS were reported, one accompanied by transverse testicular ectopia and the other associated with cryptorchidism. Corporeal hysterectomy and orchidopexy were given to both the patients and cryptorchidectory the latter.

RESULTSVascular supply and texture of the testis were normal in both the 2 patients after 1.5-2 years' follow-up.

CONCLUSIONPMDS is male pseudohermaphroditism, for which means should be taken to preserve the blood supply and fertility function of the testis in surgical management, and attention should be paid to possible development of testis tumor in follow-up.

Adult ; Disorders of Sex Development ; pathology ; Follow-Up Studies ; Humans ; Male ; Mullerian Ducts ; abnormalities ; Syndrome

OBJECTIVE: To analyze magnetic resonance imaging (MRI) findings of Mullerian remnants in young females clinically suspected of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome in a primary amenorrhea workup. MATERIALS AND METHODS: Fifteen young females underwent multiplanar T2- and transverse T1-weighted MRI at either a 1.5T or 3.0T MR imager. Two gynecologic radiologists reached consensus decisions for the evaluation of Mullerian remnants, vagina, ovaries, and associated findings. RESULTS: All cases had bilateral uterine buds in the pelvic cavity, with unilateral cavitation in two cases. The buds had an average long-axis diameter of 2.64 +/- 0.65 cm. In all cases, bilateral buds were connected with fibrous band-like structures. In 13 cases, the band-like structures converged at the midline or a paramedian triangular soft tissue lying above the bladder dome. The lower one-third of the vagina was identified in 14 cases. Fourteen cases showed bilateral normal ovaries near the uterine buds. One unilateral pelvic kidney, one unilateral renal agenesis, one mild scoliosis, and three lumbar sacralization cases were found as associated findings. CONCLUSION: Typical Mullerian remnants in MRKH syndrome consist of bilateral uterine buds connected by the fibrous band-like structures, which converge at the midline triangular soft tissue lying above the bladder dome.
Abnormalities, Multiple/*pathology ; Adolescent ; Adult ; Female ; Humans ; Kidney/abnormalities/pathology ; Magnetic Resonance Imaging/*methods ; Middle Aged ; Mullerian Ducts/abnormalities/pathology ; Retrospective Studies ; Somites/abnormalities/pathology ; Spine/abnormalities/pathology ; Uterus/abnormalities/pathology ; Vagina/abnormalities/pathology

Persistent Muellerian duct syndrome (PMDS) is a rare form of male pseudohermaphrodism without the feature of ambiguous genitalia. We present a case of PMDS with transverse testicular ectopia (TTE).
Abnormalities, Multiple ; Adult ; Disorders of Sex Development ; pathology ; surgery ; Hernia, Inguinal ; surgery ; Humans ; Male ; Mullerian Ducts ; abnormalities ; surgery ; Testicular Hydrocele ; surgery ; Testis ; abnormalities ; surgery

Congenital agenesis of the seminal vesicle (CASV) is frequently associated with congenital absence of the vas deferens (CAVD) or ipsilateral congenital vasoureteral communication. We reported two cases of a rare condition that the vas deferens open ectopically into Mullerian duct cyst associated with agenesis of the ipsilateral seminal vesicle. The diagnosis was confirmed by vasography. Transurethral unroofing of the Mullerian duct cyst was performed in both patients with favourable results, however, assisted reproductive technology (ART) was still necessary for them to father children.
Adult ; Cysts ; diagnostic imaging ; pathology ; Humans ; Infertility, Male ; diagnostic imaging ; pathology ; Male ; Mullerian Ducts ; abnormalities ; diagnostic imaging ; Seminal Vesicles ; abnormalities ; diagnostic imaging ; Tomography, X-Ray Computed ; Vas Deferens ; abnormalities ; diagnostic imaging

Radiotherapy plays a major role in the treatment of cervical cancer. A successful radiotherapy program integrates both external beam and brachytherapy components. The principles of radiotherapy are strongly based on the anatomy of the organ and patterns of local and nodal spread. However, in patients with distorted anatomy, several practical issues arise in the delivery of optimal radiotherapy, especially with brachytherapy. Müllerian duct anomalies result in congenital malformations of the female genital tract. Though being very commonly studied for their deleterious effects on fertility and pregnancy, they have not been recognized for their potential to interfere with the delivery of radiotherapy among patients with cervical cancer. Here, we discuss the management of cervical cancer among patients with Müllerian duct anomalies and review the very sparse amount of published literature on this topic.
Brachytherapy ; Diagnostic Imaging ; Female ; Genital Diseases, Female ; diagnosis ; diagnostic imaging ; pathology ; Humans ; Magnetic Resonance Imaging ; Mullerian Ducts ; abnormalities ; diagnostic imaging ; pathology ; Radiography ; Radiosurgery ; Radiotherapy ; methods ; Uterine Cervical Neoplasms ; radiotherapy ; surgery

Abnormalities, Multiple ; genetics ; metabolism ; pathology ; surgery ; Actins ; metabolism ; Adolescent ; Chromosome Inversion ; Chromosomes, Human, Y ; Diagnosis, Differential ; Disorders of Sex Development ; genetics ; pathology ; Humans ; Hypospadias ; pathology ; surgery ; Male ; Mullerian Ducts ; abnormalities ; metabolism ; pathology ; surgery ; Neprilysin ; metabolism ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Receptors, Estrogen ; metabolism ; Receptors, Progesterone ; metabolism ; Syndrome