A Case Report of Patient Presenting with Huge Abdominal Seminoma and Persistent Mullerian Duct Syndrome

Öz

Persistent Mullerian Duct Syndrome is a very rare form of male pseudo hermaphroditism. It is characterized by the presence of Mullerian duct derivatives (uterus, fallopian tubes and upper two-third of vagina) in genotypically and phenotypically normal males. The most common presenting symptoms are inguinal hernia, undescended testis and transverse testicular ectopia and infertility. The risk of malignancy in testes associated with Persistent Mullerian Duct Syndrome is up to 15%. We report a 38 year-old, phenothpically male patient, presenting with huge abdominal mass. He had underwent bilateral orchiectomy due to cryptoorchidism at 10 years old. At exploration, uterus like structure was observed adherent to mass. The case explained in this report is an example of delayed recognition of Persistent Mullerian Duct Syndrome and subsequent seminoma.

Anahtar Kelimeler

• cryptoorchidism
• Persistent Mullerian Duct Syndrome
• seminoma

Dev Abdominal Seminom ve Persistan Müllerian Kanal Sendromu Olan Hasta Olgu Sunumu

Öz

Persistan Müllerian Kanal Sendromu çok nadir görülen bir erkek psödohermofroditizm nedenidir. Bu hastalık genotipik ve fenotipik olarak normal olan erkek bireylerde görülen Müllerian kanal kalıntılarının (uterus, fallopian tüpleri ve vajenin üst 2/3’ü) varlığı ile karakterizedir. Bu hastalarda en sık görülen başvuru semptomları kasık fıtığı, inmemiş testis, transvers testiküler ektopi ve infertilitedir. Persistan Müllerian Kanal Sendromu hastalarında testis malignite oranı %15’e kadar yükselmektedir. 38 yaşında erkek hasta karın ağrısı ve karında şişlik şikayeti ile hastanemize başvurdu. Özgeçmişinde inmemiş testis nedeniyle on yaşında yapılan bilateral orşiektomi ve infertilite öyküsü vardı. Ameliyat sırasında kitleye yapışık uterus benzeri yapılar olduğu görüldü. Bu olguda geç tanı almış bir Persistan Müllerian Kanal Sendromu hastası ve ona eşlik eden seminom vakası anlatılmıştır.

Anahtar Kelimeler

• kriptoorşidizm
• persistan Müllerian Kanal sendromu
• seminom

Kaynakça

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