Perry Arie, Scheithauer Bernd W, Macaulay Robert J B, Raffel Corey, Roth Kevin A, Kros Johan M
Division of Neuropathology, Washington University School of Medicine, St. Louis, Missouri 63110-1093, USA.
J Neuropathol Exp Neurol. 2002 Nov;61(11):947-55. doi: 10.1093/jnen/61.11.947.
Oligodendroglioma represents a distinct type of diffuse glioma with a relatively favorable prognosis. Although an O2A-like glial progenitor cell of origin has been suggested, a neuronal-oligodendroglial progenitor cell is also of interest, particularly because variable degrees of neuronal marker expression have been reported in typical oligodendrogliomas. We present 2 female and 2 male patients (ages 34-54) with frontal lobe oligodendrogliomas containing a) morphologically distinct collections of small round cells with hyperchromatic nuclei, b) well-formed Homer Wright-like and perivascular rosettes, and c) demonstrable neuronal differentiation by immunohistochemistry and/or electron microscopy in the rosette-associated regions. Unlike extraventricular neurocytomas, these cases featured an infiltrative growth pattern and a classic oligodendroglioma immunophenotype in non-rosette bearing portions of each tumor. FISH analysis demonstrated chromosome 1p and 19q codeletions in 3 (75%) cases, both in regions with and without rosettes. Recurrences were common, although all patients are currently alive 4 months to 13 yr from initial diagnosis. Based on clinicopathologic and genetic features, we diagnosed these tumors as oligodendrogliomas with neurocytic differentiation. However, it is unclear whether they represent a) gliomas with divergent neuronal differentiation, b) a distinctive form of glioneuronal neoplasm, or c) a reflection of glioneuronal histogenesis in oligodendrogliomas in general. In any case, their occurrence suggests a histogenetic overlap between oligodendroglioma and extraventricular neurocytoma not previously recognized.
少突胶质细胞瘤是一种具有相对较好预后的独特类型的弥漫性胶质瘤。尽管有人提出其起源于一种类似少突胶质前体细胞（O2A），但神经 - 少突胶质前体细胞也备受关注，特别是因为在典型的少突胶质细胞瘤中已报道有不同程度的神经元标志物表达。我们报告了2例女性和2例男性患者（年龄34 - 54岁），其额叶少突胶质细胞瘤包含：a）形态上不同的小圆形细胞聚集，细胞核深染；b）结构良好的霍默·赖特（Homer Wright）样和血管周围菊形团；c）通过免疫组织化学和/或电子显微镜在菊形团相关区域可证实的神经元分化。与脑室外神经细胞瘤不同，这些病例在每个肿瘤无菊形团的部分具有浸润性生长模式和经典的少突胶质细胞瘤免疫表型。荧光原位杂交（FISH）分析显示3例（75%）病例存在1号染色体短臂（1p）和19号染色体长臂（19q）缺失，在有菊形团和无菊形团的区域均有。复发很常见，尽管所有患者目前从初次诊断起已存活4个月至13年。基于临床病理和基因特征，我们将这些肿瘤诊断为具有神经细胞分化的少突胶质细胞瘤。然而，尚不清楚它们是：a）具有不同神经元分化的胶质瘤；b）一种独特形式的神经胶质神经元肿瘤；还是c）一般少突胶质细胞瘤中神经胶质神经元组织发生的一种反映。无论如何，它们的出现提示少突胶质细胞瘤和脑室外神经细胞瘤之间存在以前未被认识到的组织发生重叠。
