Medulloblastoma with extensive nodularity: a variant occurring in the very young—clinicopathological and immunohistochemical study of four cases
Medulloblastoma with extensive nodularity: a variant occurring in the very young—clinicopathological and immunohistochemical study of four cases
复制标题
广泛结节性髓母细胞瘤：一种发生在幼年的变异——四例临床病理学和免疫组织化学研究
DOI：
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发表时间：
2003
期刊：
影响因子：
--
通讯作者：
S. Shankar
中科院分区：
文献类型：
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作者：
T. Suresh;V. Santosh;T. Yasha;B. Anandh;A. Mohanty;B. Indiradevi;S. Sampath;S. Shankar
本文报告4例广泛结节性髓母细胞瘤。患者在诊断时年龄为3岁或更小。头颅CT扫描发现三名患者有多个融合结节，具有特殊的“葡萄样”结构。在所有四名患者中进行了肿瘤的近全切除。患者目前正在接受放疗和化疗。组织学上，肿瘤的特点是极端结节性与结节内均匀性和低增殖指数。结节间区细胞丰富，由未分化的有丝分裂活跃的细胞组成。Bcl-2蛋白表达在细胞区，但明显缺席nodulation.DiscussionMBEN代表一种变异，发生在非常年幼的儿童，但有良好的预后。这种良好的结果可能与其自发的神经细胞分化有关。MBEN中Bcl-2免疫反应性的模式表明，这种蛋白可能是髓母细胞瘤中神经元分化调节的关键参与者。
Case reportFour cases of medulloblastoma with extensive nodularity (MBEN) are described. The patients were 3 years of age or younger at diagnosis. Cranial CT scan disclosed multiple coalescing nodules with peculiar ‘grape-like’ architecture in three patients. A near total excision of the tumour was performed in all four patients. The patients are currently receiving radiation and chemotherapy. Histologically, the tumours were characterized by extreme nodularity with intranodular uniformity and low proliferative index. The internodular zones were extremely cellular, composed of undifferentiated, mitotically active cells. Bcl-2 protein expression was observed in the cellular zones but was distinctly absent within nodules.DiscussionMBEN represents a variant that occurs in very young children but has a good prognosis. The favourable outcome is probably related to its spontaneous neurocytic differentiation. The pattern of Bcl-2 immunoreactivity in MBENs indicates that this protein could be a key player in the regulation of neuronal differentiation in medulloblastomas.
小脑促纤维增生性髓母细胞瘤。
影响因子：
4.6
作者：
Katsetos,CD;Herman,MM;Frankfurter,A;Gass,P;Collins,VP;Walker,CC;Rosemberg,S;Barnard,RO;Rubinstein,LJ
通讯作者：
Rubinstein,LJ
DOI：
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发表时间：
1994-07
期刊：
The American journal of pathology
影响因子：
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作者：
D. Novack;S. Korsmeyer
通讯作者：
D. Novack;S. Korsmeyer