Persistent Mullerian Duct Syndrome (PMDS) is a very rare condition, with only 200 reported cases. The incidence of PMDS with concomitant testicular malignancy is even a rarer entity, occurring in only 18-33% among all PMDS cases, and only the second reported case in the Philippines, the first with concomitant NSGCT type.

 

We were presented with a 29 y.o Male patient with an unusual presentation of a large intra-abdominal mass with bilateral cryptorchidism and infertility. Ultrasound-Guided FNAB revealed Malignant GCT, Embryonal Carcinoma of testicular origin. Patient underwent neoadjuvant chemotherapy with noted Partial Response. Bilateral Orchiectomy, TAH, Bilateral Salpingectomy was then performed, with an atrophic right testis, malignant transformation of the left testis, and incidental intra-operative finding of persistent Mullerian Duct Derivatives. Histopathology results showed negative cancer cells.

In conclusion, in patients presenting with this rare constellation of findings, PMDS with testicular malignancy should be considered to aid us in addressing both malignancy and possible infertility.