Sertoli-Leydig tumor and male pseudohermaphroditism discovered during inguinal hernia surgery

A. Pallisera, R. Jorba, L. O. Zárate, J. Catalá, I. Moysset, M. Jimeno
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Abstract

The diagnosis of inguinal hernia is usually clinical and it is performed with high sensitivity and specificity. Very occasionally, it may be confused with other diseases (lymphadenopathy, testicular pathology,etc). We report a rare case of a 80-year-old woman with a clinical diagnosis of hernia, which was underwent surgery and a tumor from the hernia orifice was found. After histological analysis we discovered that the misdiagnosed hernia was actually a tumor on a rudimentary testis. After radiological, gynecological and cytogenetic assessment we obtained an unexpected diagnosis: Male psheudohermaphroditism and Sertoli-Leydigtumor (SLCT) development on the testis. Diagnostic guidance for disorders of sexual development is based almost entirely on pediatric experience and very few guidelines are available for adults. Male pseudohermaphroditism is an intersex condition in which the carriers show a phenotype that includes external female genitalia, but a male genetic and gonadal sex. SLCT are sex-cord stromal tumors which develop in ovary and very rarely in the testis, representing 0.1–0.5% of ovarian tumors and less than 0.2% of testicular tumors. Thus far 24 case have been reported in the literature in which SLCT tumor has developed on testis.
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在腹股沟疝手术中发现支持- leydig肿瘤和男性假雌雄同体
腹股沟疝的诊断通常是临床诊断,具有很高的敏感性和特异性。偶尔,它可能与其他疾病(淋巴结病、睾丸病等)混淆。我们报告一个罕见的病例,80岁的妇女临床诊断为疝气,这是接受手术和肿瘤从疝口被发现。经过组织学分析,我们发现误诊的疝气实际上是一个肿瘤在一个基本的睾丸。经过放射学,妇科和细胞遗传学评估,我们得到了一个意想不到的诊断:男性假雌雄同体和睾丸上的支持- leydigtumor (SLCT)的发展。性发育障碍的诊断指南几乎完全基于儿科经验,很少有适用于成人的指南。男性假雌雄同体是一种双性疾病,携带者表现出包括外部女性生殖器,但男性遗传和性腺性别的表型。SLCT是发生于卵巢的性索间质肿瘤,很少发生于睾丸,占卵巢肿瘤的0.1-0.5%,睾丸肿瘤的不到0.2%。迄今为止,文献报道了24例SLCT肿瘤在睾丸上发展。
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Central European Journal of Medicine
Central European Journal of Medicine 医学-医学:内科
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