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Answer 1.D. Persistent Mullerian Duct Syndrome
- After imaging raised the possibility of neoplasm of the spermatic cord, the patient underwent laparoscopic resection of the left seminal vesicle and spermatic cord, left radical orchiectomy, and placement of testicular prosthesis.
- Pathologic evaluation discovered a poorly formed seminal vesicle and ejaculatory duct, hypoplastic vas deferens and epididymis, and a fibrotic and malformed testicle.
- Additionally, a primitive tubular vagina and endocervix was found to open into a uterine like structure that connected to a primitive fallopian tube which extended to the malformed testicle.
- No ovarian tissue was discovered. The malformed testicle showed absence of Sertoli cells, with marked Leydig cell proliferation that infiltrated local skeletal muscle.
Figure 3a. Gross pathologic specimen with the primitive vagina on the left, uterine like structure centrally, and malformed testicle on the right.
For a full-size image, please click MRI pelvis 4 .
Figure 3b. Low powered microscopic view from histologic specimen shows a primitive fallopian tube.
For a full-size image, please click MRI pelvis 5 .
Question 2. What is the karyotype of Persistent Mullerian Duct Syndrome?
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