Departments of Pathology and Neurosurgery, San Bo Brain Hospital, Capital Medical University, Haidian District, Beijing, China.
The Department of Pathology, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
J Neuropathol Exp Neurol. 2020 Nov 1;79(11):1183-1192. doi: 10.1093/jnen/nlaa107.
Chordoid gliomas (CG) of the third ventricle are characterized by chordoid and glial features, but the extent of histological variations across CG is not fully understood. Herein, we report 16 consecutive cases of CG. All 16 patients had histories of headache and vision loss; their median age was 41.7 years at the surgery. Histological examination revealed typical features of CG, including cords of epithelioid cells within the mucinous stroma and lymphoplasmacytic infiltration. Two cases exhibited atypical histological features including histiocyte-like cells. PRKCA mutation was found in 14 cases, including the 2 with histiocytic features. BRAFV600E mutation was found only in the 2 cases with histiocytic features. The patients underwent gross total tumor resection without radiotherapy or chemotherapy. Three patients died between 1 and 4 months postsurgery. Only one had a recurrence. Eleven were alive at the most recent follow-up (range: 2-58 months). These data indicate that PRKCA mutation was a good diagnostic marker for CG and additionally suggest that histiocyte-like features can be present in CG in association with BRAF mutations.
第三脑室脊索样胶质瘤（CG）的特征为脊索样和神经胶质样特征，但 CG 之间的组织学变异程度尚不完全清楚。在此，我们报告了 16 例连续的 CG 病例。所有 16 例患者均有头痛和视力丧失病史；手术时的中位年龄为 41.7 岁。组织学检查显示 CG 的典型特征，包括黏液基质内的上皮样细胞索和淋巴浆细胞浸润。有两例表现出不典型的组织学特征，包括组织细胞样细胞。14 例存在 PRKCA 突变，包括 2 例具有组织细胞样特征的病例。BRAFV600E 突变仅存在于具有组织细胞样特征的 2 例中。患者接受了大体全切除肿瘤手术，未进行放疗或化疗。3 例患者在术后 1 至 4 个月内死亡。仅 1 例复发。11 例在最近的随访中存活（范围：2-58 个月）。这些数据表明 PRKCA 突变是 CG 的良好诊断标志物，此外还表明 BRAF 突变时 CG 中可能存在组织细胞样特征。
