Clin Neuropathol. 2024 Jan-Feb;43(1):23-28. doi: 10.5414/NP301577.
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 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.
脊索样胶质瘤是一种罕见的、边界清楚的胶质肿瘤，发生于成年人，主要影响女性。由于其位置，第三脑室的室管膜细胞被认为是起源细胞。它的特征是具有黏液样和炎症性基质的脊索样特征，以及反复出现的错义突变。
我们报告了两例（30 岁女性和 45 岁男性）具有相似的行为改变和头痛症状的病例。增强磁共振成像显示中线鞍上均匀强化肿块。组织病理学和免疫组织化学表现为脊索样胶质瘤，具有排列成实性模式的索状和簇状上皮样细胞。有不同数量的黏液样基质和淋巴浆细胞浸润。未见有丝分裂、坏死或脑侵犯。细胞强烈弥漫性表达胶质纤维酸性蛋白（GFAP），核甲状腺转录因子（TTF-1）弱阳性。上皮膜抗原（EMA）和 brachyury 阴性。随后，这位女士接受了大体全切除手术，术后不久死亡。男性患者接受了放疗，在 6 个月的随访后目前情况良好。
脊索样胶质瘤的罕见发生以及在影像学和形态学上的重叠，使其成为一种真正的伪装者。免疫组织化学组合中 GFAP 和 TTF-1 的表达可有助于鉴别诊断。治疗的主要方法是完全手术切除，辅助放疗越来越重要。
