Mena H, Morrison A L, Jones R V, Gyure K A
Department of Neuropathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Cancer. 2001 Jan 1;91(1):136-43.
Central neurocytomas are composed of mature neuronal elements, frequently arranged in rosettes similar to those present in pineocytomas. This suggests the possibility of similar patterns of differentiation, including photoreceptor differentiation. The authors analyzed the immunoreactivity of central neurocytomas for retinal S-antigen, neuronal, glial, and neuroendocrine markers.
Thirty-three central neurocytomas were analyzed with reference to their clinicopathologic characteristics, immunoreactivity, and the possibility that anaplastic histologic features correlated with aggressive clinical behavior.
There were 18 male and 15 female patients. The median age at diagnosis was 30 years (range, 3-69 years). All of the tumors with specified location were related to the ventricles. Thirty-two tumors were diagnosed at surgery and 1 at autopsy. Histologic features included mineralization (20 of 33), foci of necrosis (4 of 33), chronic inflammation (4 of 33), ganglion cell differentiation (1 of 33), and lipomatous differentiation (1 of 33). None of the lesions had significant nuclear pleomorphism, mitotic activity, or vascular endothelial proliferation. Immunohistochemistry included expression of synaptophysin (33 of 33), neuron specific enolase (31 of 33), S-100 protein (25 of 33), retinal S-antigen (14 of 24), somatostatin (8 of 27), glial fibrillary acidic protein (4 of 33), neurofilament protein (3 of 22), and leucine enkephalin (1 of 27). At follow-up, 15 of 23 patients were alive an average of 8.1 years (range, 0.91-35.9 years) after surgery.
Central neurocytomas behave as slowly growing neoplasms that remain confined within one or several supratentorial ventricles and are associated with long survival after surgical excision. Malignant forms with aggressive clinical behavior were not found. The neoplastic cells can express photoreceptor differentiation possibly relating central neurocytomas to pineocytomas. Adipocyte differentiation may be present, and the possibility of a relation between the central neurocytoma and cerebellar liponeurocytoma should be entertained.
中枢神经细胞瘤由成熟的神经元成分组成，常排列成类似于松果体细胞瘤中的菊形团。这提示了类似的分化模式的可能性，包括光感受器分化。作者分析了中枢神经细胞瘤对视网膜S抗原、神经元、神经胶质和神经内分泌标志物的免疫反应性。
对33例中枢神经细胞瘤的临床病理特征、免疫反应性以及间变组织学特征与侵袭性临床行为相关的可能性进行了分析。
男性患者18例，女性患者15例。诊断时的中位年龄为30岁（范围3 - 69岁）。所有明确位置的肿瘤均与脑室有关。32例肿瘤在手术时诊断，1例在尸检时诊断。组织学特征包括矿化（33例中的20例）、坏死灶（33例中的4例）、慢性炎症（33例中的４例）、神经节细胞分化（33例中的1例）和脂肪化生（33例中的1例）。所有病变均无明显的核多形性、有丝分裂活性或血管内皮细胞增殖。免疫组化显示突触素表达（33例中的33例）、神经元特异性烯醇化酶（33例中的31例）、S - 100蛋白（33例中的25例）、视网膜S抗原（24例中的14例）、生长抑素（27例中的8例）、胶质纤维酸性蛋白（33例中的4例）、神经丝蛋白（22例中的3例）和亮氨酸脑啡肽（27例中的1例）。随访时，23例患者中的15例术后平均存活8.1年（范围0.91 - 35.9年）。
中枢神经细胞瘤表现为生长缓慢的肿瘤，局限于一个或几个幕上脑室，手术切除后生存期长。未发现具有侵袭性临床行为的恶性形式。肿瘤细胞可表达光感受器分化，这可能将中枢神经细胞瘤与松果体细胞瘤联系起来。可能存在脂肪细胞分化，应考虑中枢神经细胞瘤与小脑脂肪神经细胞瘤之间存在关联的可能性。
