Persistent Mullerian 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 Mullerian remnants

Persistent Mullerian 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 Mullerian remnants
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DOI:
10.1111/j.1464-410x.2012.11184.x
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发表时间:
2012-12-01
期刊:
影响因子:
4.5
通讯作者:
Hennayake, Supul
Hennayake, Supul
中科院分区:
医学2区
文献类型:
--
作者:
Farikullah, Jasmin;Ehtisham, Sarah;Hennayake, Supul

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描述8例持续性苗勒管综合征(PMDS)患者的临床表现和治疗,回顾性分析2001年至2011年期间8例PMDS患者的住院记录。对PMDS和苗勒管恶性肿瘤进行了广泛的PubMed检索。例PMDS和恶性苗勒管残余被确定。从我们自己的PMDS系列:5例男性双侧隐睾,3例单侧隐睾。我们发现可以通过腹腔镜切除苗勒管残余,3例患者同时进行了腹腔镜苗勒管结构切除和腹腔镜睾丸固定术。苗勒管结构可以在最小的手术创伤和未来没有恶性肿瘤风险的情况下完成。外科医生应该考虑在可能的情况下切除苗勒管残余。
OBJECTIVESTo describe the presentation and management of eight patients with persistent Mullerian duct syndrome (PMDS) seen over a 10-year period at our tertiary centre.To review the literature of Mullerian malignancies reported in PMDS.PATIENTS AND METHODSThe hospital records of eight patients with PMDS were retrospectively reviewed between 2001 and 2011.Extensive PubMed searches for PMDS and Mullerian malignancy were performed.RESULTSEleven cases with PMDS and malignancy of the Mullerian remnants were identified.From our own PMDS series: five males presented with bilateral undescended testes and three had unilateral undescended testis.We found that the Mullerian remnants could be removed by laparoscopy and three patients had simultaneous laparoscopic removal of the Mullerian structures and laparoscopic orchidopexy.CONCLUSIONSThe principle aim of orchidopexy with simultaneous laparoscopic removal of the Mullerian structures can be accomplished with minimal surgical trauma and the benefit of no malignancy risk in the future.Surgeons should consider excision of the Mullerian remnants where possible.