Suresh T N, Santosh V, Yasha T C, Anandh B, Mohanty A, Indiradevi B, Sampath S, Shankar S K
Department of Neuropathology, National Institute of Mental Health and Neurosciences, 560 029, Bangalore, India.
Childs Nerv Syst. 2004 Jan;20(1):55-60. doi: 10.1007/s00381-003-0855-5. Epub 2003 Dec 5.
Four 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.
MBEN 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.
描述了4例具有广泛结节性的髓母细胞瘤（MBEN）。这些患者在诊断时年龄为3岁或更小。头颅CT扫描显示3例患者有多个融合结节，具有独特的“葡萄样”结构。所有4例患者均接受了肿瘤近全切除。患者目前正在接受放疗和化疗。组织学上，肿瘤的特征是具有极端结节性，结节内细胞均匀且增殖指数低。结节间区域细胞极度丰富，由未分化的、有丝分裂活跃的细胞组成。在细胞区域观察到Bcl-2蛋白表达，但结节内明显缺乏。
MBEN是一种发生于非常年幼儿童的变异型，但预后良好。良好的预后可能与其自发的神经细胞分化有关。MBEN中Bcl-2免疫反应模式表明，该蛋白可能是髓母细胞瘤神经元分化调节中的关键因子。
