Vallat-Decouvelaere A V, Gauchez P, Varlet P, Delisle M B, Popovic M, Boissonnet H, Gigaud M, Mikol J, Hassoun J
Department of Pathology,Lariboisière Hospital, Paris, France.
J Neurooncol. 2000 Jun;48(2):161-72. doi: 10.1023/a:1006494308864.
Central neurocytoma (CN) is described as a rare intra-ventricular benign neuronal tumor of the brain. Two primary tumors first diagnosed as malignant and extra-ventricular neurocytomas are reported here. Histologically, the tumor of the first patient, a forty-one-year-old man, consisted of monotonous cells with round nuclei, but no fibrillar background. The second tumor, in a nineteen-year-old girl, showed areas of moderately pleomorphic round cells, with numerous rosettes and ganglion cell differentiation, in an abundant fibrillary network. Both presented calcifications. Mitoses were more frequent in recurrences and spinal locations than in the primaries. All tumors stained strongly for synaptophysin, and GFAP was partly positive in the first case only. Patients received post-surgical radiotherapy and were still alive eight and six years, respectively, after initial surgery. The interpretation of atypical cases, such as ours is not easy: the diagnoses finally retained were oligodendroglioma in the first case and ganglioneuroblastoma in the second case. Furthermore, neurocytomas atypical either by their unusual topographical or histological presentation or by their poor prognosis, have been frequently entitled in this way on synaptophysin positivity. So, we were prompted to reassess the entity of CN, seventeen years after the first description, to re-appreciate the reality of anatomo-clinical variants and to discuss the value of synaptophysin positivity in these tumors. In conclusion, it seems preferable to individualize true classical CN, which has a favorable outcome, from so-called extra-ventricular, atypical and anaplastic, clinically malignant neurocytomas for which complementary treatment is required.
中枢神经细胞瘤（CN）被描述为一种罕见的脑室内良性神经元肿瘤。本文报告了两例最初被诊断为恶性和脑室外神经细胞瘤的原发性肿瘤。组织学上，第一例患者为一名41岁男性，肿瘤由核呈圆形的单一细胞组成，但无纤维背景。第二例肿瘤发生在一名19岁女孩身上，在丰富的纤维网络中可见中度多形性圆形细胞区域，有大量菊形团和神经节细胞分化。两者均有钙化。有丝分裂在复发和脊髓部位比在原发部位更常见。所有肿瘤突触素染色均呈强阳性，仅第一例病例中胶质纤维酸性蛋白（GFAP）部分呈阳性。患者接受了术后放疗，初次手术后分别存活了8年和6年。对我们这样的非典型病例进行解读并不容易：第一例最终诊断为少突胶质细胞瘤，第二例为神经节神经母细胞瘤。此外，神经细胞瘤因其不寻常的部位或组织学表现或预后不良而不典型，常因突触素阳性而被如此命名。因此，在首次描述17年后，我们促使重新评估CN的实体，重新认识解剖临床变异的实际情况，并讨论突触素阳性在这些肿瘤中的价值。总之，将预后良好的真正经典CN与所谓的脑室外、非典型和间变性临床恶性神经细胞瘤区分开来似乎更为可取，后者需要辅助治疗。
