脐索状胶质瘤是一种罕见的、周围清晰的成人胶质肿瘤，主要影响女性。基于其位置，已提出第三脑室的纤毛细胞可能是起源细胞。其特点是具有脐索状特征，有黏液和炎性基质，并伴有复发性的PRKCA p.D463H错义突变。我们报告了两例相似主诉为行为改变和头痛的病例（30岁女性和45岁男性）。造影磁共振成像显示了中线上鞍区均匀增强的肿块。组织病理学和免疫组化检查表明其特点为脐索状胶质瘤，排列着固体模式的上皮样细胞的串珠状和团状排列。有不同数量的黏液基质和淋巴浆细胞浸润。未见有有丝分裂、坏死或脑侵润。细胞对胶质纤维酸性蛋白（GFAP）呈强阳性和弱核内甲状腺转录因子（TTF-1）呈阳性。上皮膜抗原（EMA）和短尾蛋白（brachyury）阴性。随后，该女患者接受了完全切除手术，并在手术后不久去世。该男性患者接受了放疗，目前在随访6个月后情况良好。由于脐索状胶质瘤的罕见发生率以及放射学和形态学上的重叠，使其成为一个真正的伪装者。免疫组化检查中GFAP和TTF-1的组合在鉴别诊断中是有用的。治疗的主要方法是完全切除术，辅以放射治疗的重要性正在逐步增加。

Chordoid glioma is a rare well-circumscribed glial neoplasm arising in adults and predominantly affects females. Tanycytes of the third ventricle have been proposed as the cell of origin owing to its location. It is characterized by chordoid features with myxoid and inflammatory stroma and recurrent PRKCA p.D463H missense mutation.We present two cases (30-year-old female and 45-year-old male) with similar complaints of behavioral change and headache. Midline suprasellar homogeneously enhancing mass was seen on contrast-enhanced magnetic resonance imaging. Histopathology and immunohistochemistry was characteristic of chordoid glioma with cords and clusters of epithelioid cells arranged in a solid pattern. There were variable amounts of myxoid stroma and lymphoplasmacytic infiltrate. No mitosis, necrosis, or brain invasion was noted. The cells expressed strong diffuse positivity for glial fibrillary acid protein (GFAP) and weak nuclear thyroid transcription factor (TTF-1). Epithelial membrane antigen (EMA)and brachyury were negative. Subsequently, the lady underwent gross total excision and died soon after the operation. The male patient received radiotherapy and is currently doing well after 6 months of follow-up.The rare occurrence as well as the radiological and morphological overlaps in chordoid gliomas make them a true masquerader. Combination of GFAP and TTF-1 in the immunohistochemical panel can be useful in differential diagnosis. Mainstay of treatment is complete surgical excision, with adjuvant radiotherapy becoming increasingly important.