Radke Josefine, Gehlhaar Claire, Lenze Dido, Capper David, Bock Alexander, Heppner Frank L, Jödicke Andreas, Koch Arend
Clin Neuropathol. 2015 Jan-Feb;34(1):19-25. doi: 10.5414/NP300783.
Cerebellar liponeurocytoma, first recognized as a distinct entity in the revised WHO classification of Tumors of the Central Nervous System in 2000, is a rare tumor with characteristic histological features and predominant location in the cerebellum. The proliferative index is usually low, and previous reports supported a favorable prognosis. We report a case of a second recurrence of a cerebellar liponeurocytoma with increased proliferative and mitotic activity in which extensive immunohistochemical characterization and genetic profiling were performed. The tumor specimen was characterized in terms of genetic changes frequently associated with gliomas and medulloblastomas. Considering the low number of reported cases, the prognosis of cerebellar liponeurocytoma seems difficult to assess. Our case suggests the existence of different histological grades of cerebellar liponeurocytoma and its possible progression towards a dedifferentiated, malignant phenotype, which has not yet been adequately taken into consideration in the current WHO classification.
小脑脂肪神经细胞瘤于2000年在世界卫生组织中枢神经系统肿瘤分类修订版中首次被确认为一种独特的实体肿瘤，是一种罕见肿瘤，具有特征性组织学特征，主要位于小脑。其增殖指数通常较低，既往报道提示预后良好。我们报告1例小脑脂肪神经细胞瘤二次复发，伴有增殖和有丝分裂活性增加，对其进行了广泛的免疫组化特征分析和基因谱分析。该肿瘤标本根据与胶质瘤和髓母细胞瘤常见相关的基因变化进行了特征描述。鉴于报道的病例数量较少，小脑脂肪神经细胞瘤的预后似乎难以评估。我们的病例提示存在不同组织学分级的小脑脂肪神经细胞瘤，且其可能进展为去分化的恶性表型，而这在当前世界卫生组织分类中尚未得到充分考虑。
