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老年完全性雄激素不敏感患者的恶性睾丸间质细胞瘤:一例报告

Malignant Leydig Cell Tumor in Elderly Complete Androgen Insensitivity Patient: A Case Report.

作者信息

Shrestha Sundar, Banepali Niroj, Sthapit Rakesh, Agrawal Dipesh

机构信息

Department of General Surgery, Bir hospital, Kathmandu, Nepal.

出版信息

JNMA J Nepal Med Assoc. 2019 Mar-Apr;57(216):127-129. doi: 10.31729/jnma.4206.

Abstract

There are various causes of primary amenorrhea in phenotypically females such as, complete androgen insensitivity syndrome, pure gonadal dysgenesis, 17b-hydroxysteroid dehydrogenase deficiency, or mixed gonadal dysgenesis. Primary amenorrhea in a phenotypically female is commonly encountered in Androgen Insensitivity Syndrome. In patients with Androgen Insensitivity Syndrome, with intra-abdominal testis there is high chances of developing testicular tumour, among them Sertoli cell tumour and seminoma being the most common types. Leydig cell tumour in androgen insensitivity syndrome, is very rare and malignant leydig cell tumour is even further rarer. There are few cases reported in the literature of malignant leydig cell tumour with complete androgen insensitivity. Here we are reporting a case of 65 years married elderly patient with malignant leydig cell tumour with complete androgen insentivity syndrome. Keywords: complete androgen insensitivity syndrome; leydig cell tumour; testicular feminization.

摘要

在表型为女性的患者中,原发性闭经有多种原因,如完全雄激素不敏感综合征、单纯性腺发育不全、17β-羟类固醇脱氢酶缺乏或混合性性腺发育不全。在表型为女性的患者中,原发性闭经在雄激素不敏感综合征中较为常见。在雄激素不敏感综合征患者中,若睾丸位于腹腔内,则发生睾丸肿瘤的几率较高,其中最常见的类型是支持细胞瘤和精原细胞瘤。雄激素不敏感综合征中的间质细胞瘤非常罕见,而恶性间质细胞瘤更为罕见。文献中报道的完全雄激素不敏感伴恶性间质细胞瘤的病例很少。在此,我们报告一例65岁已婚老年患者,患有完全雄激素不敏感综合征伴恶性间质细胞瘤。关键词:完全雄激素不敏感综合征;间质细胞瘤;睾丸女性化

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf96/8827592/ef5f02c13371/JNMA-57-216-127-g1.jpg

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本文引用的文献

1
Complete androgen insensitivity syndrome with a Sertoli-Leydig cell tumor.完全性雄激素不敏感综合征合并支持-间质细胞瘤
J Pediatr Adolesc Gynecol. 2014 Oct;27(5):e113-5. doi: 10.1016/j.jpag.2013.08.007. Epub 2013 Dec 14.
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Ann Endocrinol (Paris). 2008 Jun;69(3):218-26. doi: 10.1016/j.ando.2008.02.025. Epub 2008 May 6.
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Leydig cell tumors of the testis. A clinicopathological analysis of 40 cases and review of the literature.
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