González-Cámpora R, Weller R O
Departamento de Anatomía Patológica, Hospital Universitario Virgen Macarena, Facultad de Medicina de Sevilla, Spain.
Neuropathol Appl Neurobiol. 1998 Oct;24(5):397-402. doi: 10.1046/j.1365-2990.1998.00139.x.
We describe a case of a lipidized cerebellar tumour in a 67-year-old woman with immunocytochemical evidence of neurocytic, astrocytic, and in addition, striated muscle cell differentiation. A review of the literature on lipidized cerebellar tumours shows that they present in adults (mean age 58.8 years); they tend to occur in the hemispheric or paramedian regions of the cerebellum and are hypodense on CT scan and MRI. Histologically they are well differentiated and share some common features, such as extensive lipidization, low mitotic rate and an absence of pleomorphism, necrosis and capillary endothelial proliferation. The present case emphasizes the occurrence of astrocytic and striated muscle cell components in these tumours. Although the number of reported cases is limited they appear to have a better prognosis than the conventional form of adult medulloblastoma. It seems therefore that the term lipidized mature neuroectodermal tumour, rather than lipidized medulloblastoma, would be a more appropriate term for these tumours.
我们描述了一例67岁女性的脂质化小脑肿瘤病例，其具有神经细胞、星形细胞以及横纹肌细胞分化的免疫细胞化学证据。对脂质化小脑肿瘤的文献回顾显示，它们多见于成年人（平均年龄58.8岁）；倾向于发生在小脑半球或旁正中区域，在CT扫描和MRI上呈低密度。组织学上，它们分化良好，具有一些共同特征，如广泛的脂质化、低有丝分裂率以及无多形性、坏死和毛细血管内皮增殖。本病例强调了这些肿瘤中星形细胞和横纹肌细胞成分的出现。尽管报告的病例数量有限，但它们的预后似乎比成人髓母细胞瘤的传统形式要好。因此，对于这些肿瘤，“脂质化成熟神经外胚层肿瘤”这一术语似乎比“脂质化髓母细胞瘤”更为合适。
