Department of Experimental and Clinical Neuropathology, M. Mossakowski Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland.
Brain Tumor Pathol. 2011 Jul;28(3):265-71. doi: 10.1007/s10014-011-0036-2. Epub 2011 May 12.
Lipidization is observed only occasionally in primary neuroectodermal tumors of the central nervous system. It may reflect lipomatous transformation of tumor cells into xanthomatous and/or adipocyte-like cells. We report a unique case of mixed glioneuronal tumor with marked lipomatous changes in a young patient with intractable epilepsy. MRI revealed a well-circumscribed lesion in the right temporal lobe. Histopathological findings showed the pleomorphic tumor with numerous cells containing large lipid droplets, resembling mature adipocytes, that were arranged in clusters or scattered within the neoplastic tissue. The tumor was composed of both glial and neuronal elements. Some tumor cells displayed features intermediate between glial and neuronal cells. The reticulin fibers were limited to blood vessels. Mitotic figures, vascular proliferation, and necrosis were absent, and MIB-1 labeling index was less than 1%. Diffuse immunoreactivity for GFAP and S100-protein was observed. In some heavily lipidized cells, the lipid droplets were surrounded by a cytoplasmic rim of GFAP immunoreactivity. Numerous cells exhibited immunostaining for NSE and synaptophysin. This is the first documented case of glioneuronal tumor with extensive lipomatous transformation, which might be considered as a heavily lipidized unclassified pleomorphic glioneuronal tumor or a variant of lipoganglioglioma with marked pleomorphism and severe lipidization.
脂质化仅偶尔见于中枢神经系统原发性神经外胚层肿瘤。它可能反映了肿瘤细胞向脂肪细胞、黄瘤细胞和/或脂肪细胞样细胞的脂肪化生。我们报告了一例年轻难治性癫痫患者具有明显脂肪化生的混合性神经胶质神经元肿瘤的独特病例。MRI 显示右颞叶边界清楚的病变。组织病理学发现显示多形性肿瘤，含有大量脂滴的细胞数量众多，类似于成熟的脂肪细胞，这些细胞呈簇状或散在排列在肿瘤组织中。肿瘤由神经胶质和神经元成分组成。一些肿瘤细胞表现出介于神经胶质细胞和神经元细胞之间的特征。网状纤维仅限于血管。无有丝分裂象、血管增生和坏死，MIB-1 标记指数小于 1%。弥漫性 GFAP 和 S100 蛋白免疫反应阳性。在一些脂质化严重的细胞中，脂滴被 GFAP 免疫反应的细胞质边缘包围。许多细胞对 NSE 和突触素呈免疫反应。这是首例广泛脂肪化生的神经胶质神经元肿瘤的记录病例，可被视为高度脂质化的未分类多形性神经胶质神经元肿瘤或伴有明显多形性和严重脂质化的脂肪性神经节胶质瘤的变体。
