Chaturvedi Sujata, Gupta Sanjeev, Singhal Shweta, Kumari Rima
Department of Pathology, Institute of Human Behavior and Allied Sciences, Delhi, India.
Neuropathology. 2008 Feb;28(1):93-7. doi: 10.1111/j.1440-1789.2007.00833.x.
Medulloblastoma containing a striated muscle component is a rare embryonal cerebellar neoplasm, designated as medullomyoblastoma. We report here a case of medullomyoblastoma in a 9-year-old boy who developed signs of increased intracranial pressure and truncal ataxia. MRI disclosed a well-defined, egg-sized mass with heterogenous intermediate signal intensity in the cerebellar vermis, which compressed the 4th ventricle. The resected tumor consisted of hyperchromatic, undifferentiated neuroectodermal cells and desmin-positive myogenic cells with striation, compatible with medullomyoblastoma. MIB labeling index was 8% in undifferentiated cells but no labeling was seen in the myogenic cells. In addition, there were areas of calcification and psammoma bodies, which was a rare finding in this tumor.
含有横纹肌成分的髓母细胞瘤是一种罕见的胚胎性小脑肿瘤,称为髓肌母细胞瘤。我们在此报告一例9岁男孩的髓肌母细胞瘤病例,该男孩出现颅内压升高和躯干共济失调症状。MRI显示在小脑蚓部有一个边界清晰、鸡蛋大小的肿块,信号强度不均匀呈中等,压迫第四脑室。切除的肿瘤由深染的未分化神经外胚层细胞和有横纹的结蛋白阳性肌源性细胞组成,符合髓肌母细胞瘤。未分化细胞的MIB标记指数为8%,但肌源性细胞未见标记。此外,还有钙化区和砂粒体,这在该肿瘤中是罕见的发现。