Pedram Mohammad, Vafaie Majid, Fekri Kiavash, Haghi Sabahat, Rashidi Iran, Pirooti Chia
Thalassemia and Hemoglobinopathy Research Center, Ahvaz Jondishapur University of Medical Sciences, Ahvaz, Iran.
Dept. of Pathology, Shafa Hospital , Ahvaz Jondishapur University of Medical Sciences, Ahvaz, Iran.
Iran J Cancer Prev. 2013 Summer;6(3):174-6.
Neuroblastoma is the third most common malignancy of childhood, after leukemia and brain tumors. Only 2% of all neuroblastoma occur in the brain. Primary cerebellar neuroblastoma is an specific subset of Primitive Neuroectodermal Tumors (PNET). Meduloblastoma is a relatively common and well-established entity, consisting of primitive and multipotential cells that may exhibit some evidence of neuroblastic or gliad differentiation. But cerebellar neuroblastoma with ultrastractural evidence of significant neuroblastic differentiation is extremely rare. We report a rare case of neuroblastoma in the cerebellum. A 2.5-year-old Iranian boy presented with vomiting and nausea in the morning and ataxia. CT scan showed a tumor mass in the cerebellum and the report of radiologist was medulloblastoma. Light microscopic assay showed a small cell neoplasm with lobules of densely packed cells (lobulated pattern) and better differentiated cells. Neuron-Specific Enolase was positive. Pathologic diagnosis confirmed the existence of cerebellar neuroblastoma. Chemotherapy followed surgical removal. No relapse occurred 12 months after surgery.
神经母细胞瘤是儿童期第三常见的恶性肿瘤，仅次于白血病和脑肿瘤。所有神经母细胞瘤中仅有2%发生于脑部。原发性小脑神经母细胞瘤是原始神经外胚层肿瘤（PNET）的一个特殊亚型。髓母细胞瘤是一种相对常见且已明确的实体瘤，由原始的多能细胞组成，这些细胞可能表现出一些神经母细胞或神经胶质分化的证据。但具有超微结构证据显示显著神经母细胞分化的小脑神经母细胞瘤极为罕见。我们报告一例小脑神经母细胞瘤的罕见病例。一名2.5岁的伊朗男孩晨起出现呕吐、恶心及共济失调。CT扫描显示小脑有一肿瘤块，放射科医生报告为髓母细胞瘤。光镜检查显示为小细胞肿瘤，有紧密排列的细胞小叶（小叶状模式）及分化较好的细胞。神经元特异性烯醇化酶呈阳性。病理诊断证实为小脑神经母细胞瘤。手术切除后进行化疗。术后12个月未出现复发。
