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老年患者手术中诊断出睾丸女性化综合征。病例报告。

Testicular feminization syndrome diagnosed in an elderly patient at surgery. A case report.

作者信息

Monaco A, La Brocca A, Verna C, Moncelli G, Berardengo E, Bollito E

机构信息

Department of General Surgery, Civil Hospital of Giaveno, Turin, Italy.

出版信息

Panminerva Med. 1996 Sep;38(3):193-8.

PMID:9009687
Abstract

A case of Morris' syndrome in which the diagnosis has been realized only in old age is reported. A 69 year-old patient, with female external genitalia and secondary sexual characteristics, was referred to us with a diagnosis of a mass in the right inguinal region. Her personal history was based on a primary amenorrhoea, which was unsuccessfully investigated since she was adolescent. At the age of 63, during surgery for a left inguinal hernia realized in another hospital, a testis-like mass with the spermatic cord was casually found. During our hospitalization, a surgical removal of the right inguinal mass was performed, and the histologic examination showed the presence of a dominant sclerohyalin testicular tissue without evidence of seminal epithelium and sparse focuses of Leydig cells hyperplasia. Besides, the determination of gonadotropins and sex hormones yielded an increased production of LH, FSH, estradiol, testosterone and androstenedione. A cytogenetic analysis showed a 46, XY karyotype. The diagnosis realized only in old age has compelled the patient to live all her life, from sexual maturity, with indecision and doubt, and without a clinical explanation of fundamental utility even from the psychological point of view. Finally, in our patient the absence of cytologic aspect of malignant transformation in the removed testes in a six years period, seem fortuitous. It is always necessary to consider Morris' syndrome among the possible diseases causing primary amenorrhoea in the clinical evaluation of young phenotypic female patients.

摘要

报告了一例仅在老年时才确诊的莫里斯综合征病例。一名69岁患者,具有女性外生殖器和第二性征,因右侧腹股沟区肿物诊断被转诊至我院。她的个人病史始于原发性闭经,自青春期起对此进行的检查均未成功。63岁时,在另一家医院进行左侧腹股沟疝手术期间,偶然发现一个类似睾丸的肿物连带着精索。在我院住院期间,对右侧腹股沟肿物进行了手术切除,组织学检查显示存在占主导地位的硬化透明样睾丸组织,未见生精上皮,仅有散在的睾丸间质细胞增生灶。此外,促性腺激素和性激素测定显示促黄体生成素、促卵泡生成素、雌二醇、睾酮和雄烯二酮分泌增加。细胞遗传学分析显示核型为46, XY。仅在老年时才确诊,这使得患者从性成熟开始便一生都充满犹豫和怀疑,甚至从心理学角度也没有得到根本有用的临床解释。最后,在我们的患者中,切除的睾丸在六年期间未出现恶性转化的细胞学表现,这似乎是偶然的。在对年轻表型女性患者进行临床评估时,在可能导致原发性闭经的疾病中,始终有必要考虑莫里斯综合征。

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