Division of Brain Tumor Translational Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan.
Brain Tumor Pathol. 2015 Apr;32(2):131-6. doi: 10.1007/s10014-014-0198-9. Epub 2014 Aug 22.
Multinodular and vacuolating neuronal tumors of the cerebrum (MVNT) are superficial neuronal tumors in adults that were first documented in 2013. Herein, we report a case of MNVT involving a 37-year-old man who presented with an epileptogenic, superficial solid lesion in the left parietal lobe. Histomorphology of the resected specimen was characterized by nodular lesions with vacuolation. Nodules comprised irregular proliferation of neuronal cells, which ranged from ganglion-like forms to those with indistinct lineage. Immunohistochemical analysis showed that the lesional cells stained positively for HuC/HuD, synaptophysin, and Olig2, and negatively for NeuN, neurofilament, chromogranin A, GFAP, CD34, IDH1(R132H), and BRAF(V600E). Eighteen months following surgery, the patient is well and without neurological deficits. MVNTs are distinctive tumors that should be differentiated from ganglion cell tumors, dysembryoplastic neuroepithelial tumors, and malformation of cortical development.
大脑多结节性和空泡性神经元肿瘤(MVNT)是成人中一种位于脑表面的神经元肿瘤,于 2013 年首次被报道。本文报告了一例累及 37 岁男性的 MVNT 病例,该患者表现为左顶叶致痫性、脑表面实性病变。切除标本的组织形态学特征为有空泡的结节性病变。结节由神经元细胞不规则增生构成,从类似于神经节的形式到谱系不明确的形式不等。免疫组化分析显示,病变细胞 HuC/HuD、突触素和 Olig2 阳性,而 NeuN、神经丝、嗜铬粒蛋白 A、GFAP、CD34、IDH1(R132H)和 BRAF(V600E)阴性。术后 18 个月,患者情况良好,无神经功能缺损。MVNTs 是一种具有特征性的肿瘤,应与神经节细胞瘤、发育不良性神经上皮肿瘤和皮质发育畸形相鉴别。