authors
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Grzegorz Kudela, Miros³aw Mikosiñski, Wojciech Utrata, El¿bieta Kuleta-Bosak, Barbara Kalina-Faska, Tomasz Koszutski
- Oddzia³ Urologii Dzieciêcej Górno¶l±skiego Centrum Zdrowia Dziecka w Katowicach
Zak³ad Radiologii Górno¶l±skiego Centrum Zdrowia Dziecka w Katowicach
Klinika Endokrynologii Dzieciêcej Górno¶l±skiego Centrum Zdrowia Dziecka w Katowicach
keywords
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persistent müllerian duct disorders of sex development cryptorchidism
summary
- Persistent müllerian duct syndrome (PMDS) is a rare entity in which fallopian tubes, uterus and upper part of the vagina are present
- in normally virilized XY patients. Transverse testicular ectopia is often associated with persistence of müllerian remnants. The authors
- report two brothers with PMDS and review the literature. PMDS should be considered in boys with cryptorchidism. Because of the
- high risk of malignancy, the testes should be mobilized into the scrotum or into another palpable position. The müllerian structures
- left in situ should be monitored because the risk of malignancy exists although it is low.
references
- Dekker HM, de Jong IJ, Sanders J, Wolf RF: Persistent Mullerian duct syndrome. Radiographics 2003, 23 (2), 309-313.
- Ueno S, Yokoyama S, Hirakawa H: A Case of Transverse Testicular Ektopia With Müllerian Duct Remnant. J Pediatr Surg 2001, 36 (11), 1710-1712.
- Dias A, Carrillo A, Bauer M, Labie A, Danon M: Persistent Müllerian Duct Syndrom in an Infant with Initial Bilateral Criptorchidism. Int Pediatr 2000, 15, 41-43.
- Rey R: Anti-Müllerian hormone in disorders of sex determination and differentiation. Arq Bras Endocrinol Metab 2005, 49, (1), 26-36.
- Joss N: The undervirilized male child: endocrine aspects. BJU Int 2004, 93, suppl.3, 3-5.
- Lang-Muritano M, Biason-Lauber A, Gitzemann C et al: A novel mutation in the anti-müllerian hormone gene as cause of persistent müllerian duct syndrome. Eur J Pediatr 2001, 160 (11), 652-654.
- Otsuka T, Nakazano T, Matsuo Y et al: Persistent Müllerian Duct Syndrome Suggested by Magnetic Resonance Imaging. Radiation Medicine 2003, 21 (6) 277-279.
- Vandersteen DR, Chameton AK, Ireland K, Tank ES: Surgical management of persistent müllerian duct syndrome. Urology 1997, 49 (6), 941-945.
- Manassero F, Cuttano MG, Morelli G. et al: Mixed germ cell tumor after bilateral orchidopexy in persistent Mullerian duct syndrome with transverse testicular ectopia. Urol Int 2004, 73 (1), 81-83.
- Buchholz NP, Biyabani R, Herzig MJ: Persistent Mullerian duct syndrome. Eur Urol 1998, 34 (3), 230-232.
- Thiel DD, Erhard MJ: Uterine adenosarcoma in a boy with persistent mullerian duct syndrome: first reported case. J Pediatr Surg 2005, 40 (9), 29-31.
- Romero FR, Fucs M, Castro MG: Adenocarcinoma of persistent mullerian duct remnants: case report and differential diagnosis. Urology 2005, 66 (1), 194-195.
- Shinmura Y, Yokoi T, Tsutsui Y: A case of clear cell adenocarcinoma of the mullerian duct in persistent mullerian duct syndrome: the first reported case. Am J Surg Pathol 2002, 26 (9), 1231-1234.
correspondence
Grzegorz Kudela
Oddzia³ Urologii Dzieciêcej
Górno¶l±skiego Centrum Zdrowia Dziecka
ul. Medyków 16
40-752 Katowice
tel. (032) 207 18 37
kudela@mp.pl
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