Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Weill Medical College of Cornell University, Houston, 77030, TX.
Department of Urology, Houston Methodist Hospital, Houston, 77030, TX.
Hum Pathol. 2018 May;75:125-131. doi: 10.1016/j.humpath.2017.10.033. Epub 2017 Nov 12.
Persistent müllerian duct syndrome (PMDS) is a form of disordered sex development in which rudimentary müllerian structures are identified in phenotypically and genotypically normal males. It is caused by defects in the anti-müllerian hormone (AMH) system. Since patients with PMDS present with undescended testes, testosterone production by Leydig cells later in life is often decreased. The role of androgens in prostate cancerogenesis is well known. Cryptorchid testes and diminished testosterone levels in post-pubertal life in patients with PMDS play a protective role against prostate cancer, and hence, prostate cancer is a rare event in patients with PMDS. Herein, we present a patient who underwent prostatectomy for high-grade prostatic adenocarcinoma with persistent müllerian structures (such as rudimentary uterus, fallopian tubes, and cervix) identified during surgery. To our knowledge, this is the second case reported in the English language literature where PMDS was associated with prostate cancer.
持续性苗勒管综合征(PMDS)是一种性发育障碍,表现为表型和基因型正常的男性存在原始苗勒管结构。它是由抗苗勒管激素(AMH)系统缺陷引起的。由于 PMDS 患者存在隐睾,因此 later 在生活中 Leydig 细胞产生的睾酮通常会减少。雄激素在前列腺癌发生中的作用是众所周知的。隐睾和 PMDS 患者青春期后睾酮水平降低对前列腺癌起到保护作用,因此 PMDS 患者中前列腺癌很少见。在此,我们介绍了一位患者,他因高级别前列腺腺癌接受了前列腺切除术,术中发现存在持续性苗勒管结构(如原始子宫、输卵管和宫颈)。据我们所知,这是英语文献中第二例报道的 PMDS 与前列腺癌相关的病例。