Shrestha Sundar, Banepali Niroj, Sthapit Rakesh, Agrawal Dipesh
Department of General Surgery, Bir hospital, Kathmandu, Nepal.
JNMA J Nepal Med Assoc. 2019 Mar-Apr;57(216):127-129. doi: 10.31729/jnma.4206.
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.
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