LARGE-CELL MEDULLOBLASTOMAS - A DISTINCT VARIANT WITH HIGHLY AGGRESSIVE-BEHAVIOR
LARGE-CELL MEDULLOBLASTOMAS - A DISTINCT VARIANT WITH HIGHLY AGGRESSIVE-BEHAVIOR
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DOI：
10.1097/00000478-199207000-00007
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发表时间：
1992-07-01
影响因子：
5.6
通讯作者：
MONTALDI, A
中科院分区：
文献类型：
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作者：
GIANGASPERO, F;RIGOBELLO, L;MONTALDI, A
我们报告四例婴儿小脑肿瘤，其细胞由大的囊状核和突出的核仁组成。4例突触素均呈强阳性反应，1例呈神经丝阳性反应。N-myc和c-myc癌基因的筛选杂交显示1例c-myc扩增27倍。本例细胞遗传学分析显示双分钟等位染色体17q。从其中一个肿瘤中获得的裸鼠脑内异种移植瘤显示出与原始肿瘤相似的形态以及突触素和神经细丝的基质免疫反应。所有肿瘤的特点都是高度侵袭性的行为，导致早期脑脊液扩散，尽管放疗和化疗。我们的结论是，大细胞髓母细胞瘤是髓母细胞瘤的一种独特且更具侵袭性的变种，需要更积极的治疗。
We present four cases of infantile cerebellar neoplasms composed of cells with large vesicular nuclei with prominent nucleoli. All four cases were strongly immunoreactive for synaptophysin, and one case showed immunoreactivity for neurofilaments. Filter hybridization for N-myc and c-myc oncogenes showed a 27-fold c-myc amplification in one case. The cytogenetic analysis in this case showed Double-Minutes and isochromosome 17q. An intraccrebral xenograft in nude mice obtained from one such tumor showed a similar morphology to that of the original tumor as well as stromg immunoreactivity for synaptophysin and neurofilaments. All the neoplasms were characterized by highly aggressive behavior leading to early cerebrospinal fluid dissemination despite radiotherapy and chemotherapy. We conclude that large-cell medulloblastoma represents a distinct and more aggressive variant of medulloblastoma that requires more aggressive therapy.