Bergmann M, Pietsch T, Herms J, Janus J, Spaar H J, Terwey B
Institut für klinische Neuropathologie, Zentralkrankenhaus Bremen-Ost, Germany.
Acta Neuropathol. 1998 Feb;95(2):205-12. doi: 10.1007/s004010050788.
Medullomyoblastoma is a rare variant of medulloblastoma containing myoblastic elements. A 9-year-old boy developed a cerebellar syndrome and signs of increased intracranial pressure, the cause of which was a tumor of the cerebellar vermis measuring 7 x 4.5 x 4.5 cm. Morphologically the tumor largely consisted of a medulloblastoma component but displayed glial, myoblastic and ganglionic differentiation on light microscopic, immunohistochemical and ultrastructural examination. The non-enhancing rim of the tumor on magnetic resonance imaging showed extensive ganglionic differentiation. The tumor did not express bcl-2, c-myc, or c-erb-B2 oncoproteins and was negative for the p53 gene product. On molecular genetic studies, the tumor did not show allelic loss on chromosome loci, frequently altered in medulloblastomas, such as 17p, 1q and 9q.
髓肌母细胞瘤是髓母细胞瘤的一种罕见变体,含有成肌细胞成分。一名9岁男孩出现小脑综合征和颅内压升高的体征,病因是小脑蚓部有一个大小为7×4.5×4.5厘米的肿瘤。形态学上,肿瘤主要由髓母细胞瘤成分组成,但在光镜、免疫组化和超微结构检查中显示出神经胶质、成肌细胞和神经节分化。磁共振成像上肿瘤的无强化边缘显示出广泛的神经节分化。肿瘤不表达bcl-2、c-myc或c-erb-B2癌蛋白,且p53基因产物为阴性。在分子遗传学研究中,该肿瘤在髓母细胞瘤中经常发生改变的染色体位点,如17p、1q和9q上未显示等位基因缺失。