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
Kaynakça
- Farikullah J, Ehtisham S, Nappo S, Patel L, Hennayake S. Persistent Müllerian duct syndrome: lessons learned from managing a series of eight patients over a 10-year period and review of literature regarding malignant risk from the Müllerian remnants. BJU Int. 2012;110(11 Pt C):E1084-E1089.
- Kaul A, Srivastava KN, Rehman SM, Goel V, Yadav V. Persistent Müllerian duct syndrome with transverse testicular ectopia presenting as an incarcerated inguinal hernia. Hernia. 2011;15(6):701-704.
- Rey RA, Grinspon RP. Normal male sexual differentiation and aetiology of disorders of sex development. Best Pract Res Clin Endocrinol Metab. 2011;25(2):221-238.
- Alp BF, Demirer Z, Gürağaç A, et al. Persistent Mullerian duct syndrome with transverse testicular ectopia and seminoma. Int Urol Nephrol. 2014;46(8):1557-1562.
- Imbeaud S, Carré-Eusèbe D, Rey R, Belville C, Josso N, Picard JY. Molecular genetics of the persistent müllerian duct syndrome: a study of 19 families. Hum Mol Genet. 1994;3(1):125-131.
- Yamada K, Takahata A, Ichijo Y, et al. A case of testicular seminoma in persistent Mullerian duct syndrome with transverse testicular ectopia. Abdom Imaging. 2015;40(3):475-479.
- Belville C, Josso N, Picard JY. Persistence of Müllerian derivatives in males. Am J Med Genet. 1999;89(4):218-223.
- Rehman A, Hasan Z, Amanat S, et al. Combined persistent Mullerian Duct Syndrome, Transverse Testicular Ectopia and Mosaic Klinefelter's Syndrome. J Coll Physicians Surg Pak. 2008;18(6):375-377.
- Rane SR, Dangmali DP, Vishwasrao SD, Puranik SC. Persistent Mullerian Duct Syndrome with Testicular Seminoma in Transverse Testicular Ectopia. J Hum Reprod Sci. 2018;11(3):300-302.
- Sarı A, Bölükbaşı A, Kuşku F, Ermete M. Bilateral Seminoma Arising in Transverse Testicular Ectopia with Persistent Müllerian Duct Syndrome and its Metastasis to Rudimentary Uterus: Case Report. Turkiye Klinikleri Journal of Medical Sciences, 2011. 31(4): p. 1015-1018.
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
Kaynakça
- Farikullah J, Ehtisham S, Nappo S, Patel L, Hennayake S. Persistent Müllerian duct syndrome: lessons learned from managing a series of eight patients over a 10-year period and review of literature regarding malignant risk from the Müllerian remnants. BJU Int. 2012;110(11 Pt C):E1084-E1089.
- Kaul A, Srivastava KN, Rehman SM, Goel V, Yadav V. Persistent Müllerian duct syndrome with transverse testicular ectopia presenting as an incarcerated inguinal hernia. Hernia. 2011;15(6):701-704.
- Rey RA, Grinspon RP. Normal male sexual differentiation and aetiology of disorders of sex development. Best Pract Res Clin Endocrinol Metab. 2011;25(2):221-238.
- Alp BF, Demirer Z, Gürağaç A, et al. Persistent Mullerian duct syndrome with transverse testicular ectopia and seminoma. Int Urol Nephrol. 2014;46(8):1557-1562.
- Imbeaud S, Carré-Eusèbe D, Rey R, Belville C, Josso N, Picard JY. Molecular genetics of the persistent müllerian duct syndrome: a study of 19 families. Hum Mol Genet. 1994;3(1):125-131.
- Yamada K, Takahata A, Ichijo Y, et al. A case of testicular seminoma in persistent Mullerian duct syndrome with transverse testicular ectopia. Abdom Imaging. 2015;40(3):475-479.
- Belville C, Josso N, Picard JY. Persistence of Müllerian derivatives in males. Am J Med Genet. 1999;89(4):218-223.
- Rehman A, Hasan Z, Amanat S, et al. Combined persistent Mullerian Duct Syndrome, Transverse Testicular Ectopia and Mosaic Klinefelter's Syndrome. J Coll Physicians Surg Pak. 2008;18(6):375-377.
- Rane SR, Dangmali DP, Vishwasrao SD, Puranik SC. Persistent Mullerian Duct Syndrome with Testicular Seminoma in Transverse Testicular Ectopia. J Hum Reprod Sci. 2018;11(3):300-302.
- Sarı A, Bölükbaşı A, Kuşku F, Ermete M. Bilateral Seminoma Arising in Transverse Testicular Ectopia with Persistent Müllerian Duct Syndrome and its Metastasis to Rudimentary Uterus: Case Report. Turkiye Klinikleri Journal of Medical Sciences, 2011. 31(4): p. 1015-1018.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Klinik Tıp Bilimleri |
| Bölüm | Olgu Sunumu |
| Yazarlar | |
| Yayımlanma Tarihi | 28 Şubat 2021 |
| Gönderilme Tarihi | 4 Ekim 2020 |
| Kabul Tarihi | 16 Şubat 2021 |
| Yayımlandığı Sayı | Yıl 2021 Cilt: 3 Sayı: 1 |
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