We present a 27-yr-old female with gonadal dysgenesis (46, XY), who presented to our hospital with poor consciousness, aphasia, restlessness, and visual hallucination. Physical examination revealed normal breast development and normal external female genetalia. Computed tomography scan of the head and neck revealed the presence of brain edema, hydrocephalous, and a localized hypodense lesion in the hypothalamus. Her serum was positive for the anti-Ma2, which is associated with paraneoplastic encephalitis syndrome. Computed tomography of the abdomen revealed the presence of a 7.5×5.3×3.0 cm solid pelvic mass. Interestingly, a single microscopic focus of dysgerminoma was identified in a background of stromal fibrosis and focal dystrophic calcifications. No ovarian stroma or testicular tissue was identified. To our knowledge, this is the first case of gonadal dysgenesis presenting with anti-Ma2 paraneoplastic encephalitis with dysgerminoma. A discussion about paraneoplastic encephalitis with a microscopic dysgerminoma associated with anti-Ma2 antibody is presented.

译文

我们介绍了一名患有性腺发育不全的27岁女性 (46,XY),她因意识不良,失语,躁动和视觉幻觉而到我们医院就诊。体格检查显示乳房发育正常,外部女性生殖器正常。头部和颈部的计算机断层扫描显示存在脑水肿,脑积水和下丘脑局部低密度病变。她的血清对anti-Ma2呈阳性,这与副肿瘤性脑炎综合征有关。腹部计算机断层扫描显示存在7.5 × 5.3 × 3.0厘米实性骨盆肿块。有趣的是,在基质纤维化和局灶性营养不良钙化的背景下,发现了无性细胞瘤的单个微观焦点。未发现卵巢基质或睾丸组织。据我们所知,这是第一例出现anti-Ma2副肿瘤性脑炎伴无性细胞瘤的性腺发育不全。提出了关于副肿瘤性脑炎与anti-Ma2抗体相关的微观无性细胞瘤的讨论。

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