Louis D N, Swearingen B, Linggood R M, Dickersin G R, Kretschmar C, Bhan A K, Hedley-Whyte E T
Department of Pathology, Massachusetts General Hospital, Boston.
J Neurooncol. 1990 Dec;9(3):231-8. doi: 10.1007/BF02341154.
The clinical features, pathologic findings and treatment courses of eight adults with central nervous system small-cell neuronal tumors were reviewed. Five patients had central neurocytomas, two patients central nervous system neuroblastomas, and one patient a neurocytoma-like spinal cord tumor. The neurocytomas were intraventricular, moderately cellular tumors with bland nuclei and perinuclear halos. Patients with neurocytoma were treated with surgery, radiation therapy, and/or chemotherapy, and have followed favorable clinical courses. The neuroblastomas were intraparenchymal, hypercellular tumors with necrosis and frequent mitoses. Patients with neuroblastomas were treated with surgery, radiation therapy and chemotherapy, with some clinical response, but overall poor survival. One of the two patients developed extracranial metastasis. The spinal cord tumor had histologic features of neurocytoma, and responded well to biopsy and radiation therapy. The cases are compared with the varieties of small-celled neuronal tumors described in the literature, and pathologic, histogenetic and treatment implications are discussed.
回顾了8例成人中枢神经系统小细胞神经元肿瘤的临床特征、病理表现及治疗过程。5例为中枢神经细胞瘤，2例为中枢神经系统神经母细胞瘤，1例为神经细胞瘤样脊髓肿瘤。神经细胞瘤为脑室内肿瘤，细胞中等密度，细胞核温和，有核周晕。神经细胞瘤患者接受了手术、放疗和/或化疗，临床过程良好。神经母细胞瘤为脑实质内肿瘤，细胞高度密集，有坏死和频繁的有丝分裂。神经母细胞瘤患者接受了手术、放疗和化疗，有一定临床反应，但总体生存率较差。2例患者中有1例发生颅外转移。脊髓肿瘤具有神经细胞瘤的组织学特征，对活检和放疗反应良好。将这些病例与文献中描述的各种小细胞神经元肿瘤进行了比较，并讨论了病理、组织发生及治疗意义。
