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脂肪神经细胞瘤的临床病理特征

The clinicopathological features of liponeurocytoma.

作者信息

Xu Li, Du Jiang, Wang Junmei, Fang Jingyi, Liu Zhaoxia, He Yanjiao, Li Guilin

机构信息

Department of Neuropathology, Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, China National Clinical Research Center for Neurological Diseases (NCRC-ND), Center of Brain Tumor, Beijing Institute for Brain Disorders, Beijing Key Laboratory of Brain Tumor, Beijing, 100050, China.

出版信息

Brain Tumor Pathol. 2017 Jan;34(1):28-35. doi: 10.1007/s10014-017-0279-7. Epub 2017 Feb 24.

Abstract

To discuss the clinicopathological features of liponeurocytoma, we retrospectively reviewed three liponeurocytoma cases and compared their immunophenotypes and genotypes with those of similar tumors. Furthermore, we reviewed the literature and compared the similarities and differences between cerebellar and intraventricular liponeurocytomas. Two cerebellar and one intraventricular liponeurocytomas were included in the present study. The liponeurocytomas comprised small tumor cells and lipomatous cells. The tumor cells expressed SYN, MAP-2, and NeuN. One case showed atypical histological features. By reviewing the literature, we found that cerebellar liponeurocytoma tended to be more common in females, whereas the converse was true for intraventricular liponeurocytoma. Compared with cerebellar liponeurocytoma, intraventricular liponeurocytoma was more commonly noted in younger adult patients. A high MIB-1 index (>10%) and incomplete tumor resection might represent adverse prognostic factors in patients with liponeurocytoma. We suggest that 'central liponeurocytoma' should be used to include all putative liponeurocytoma sites. The present study identified several morphological, immunohistochemical, and genetic features that may aid in the differential diagnosis of liponeurocytoma. In addition, surgery should be the preferred treatment, and complete tumor resection should be the goal. Additional cases with long-term follow-up are needed to develop optimal management protocols for liponeurocytoma.

摘要

为探讨脂肪神经细胞瘤的临床病理特征,我们回顾性分析了3例脂肪神经细胞瘤病例,并将其免疫表型和基因型与相似肿瘤进行比较。此外,我们查阅了文献,比较了小脑和脑室内脂肪神经细胞瘤之间的异同。本研究纳入了2例小脑脂肪神经细胞瘤和1例脑室内脂肪神经细胞瘤。脂肪神经细胞瘤由小肿瘤细胞和脂肪细胞组成。肿瘤细胞表达SYN、MAP-2和NeuN。1例表现出非典型组织学特征。通过查阅文献,我们发现小脑脂肪神经细胞瘤在女性中更常见,而脑室内脂肪神经细胞瘤则相反。与小脑脂肪神经细胞瘤相比,脑室内脂肪神经细胞瘤在年轻成年患者中更常见。高MIB-1指数(>10%)和肿瘤切除不完全可能是脂肪神经细胞瘤患者的不良预后因素。我们建议“中枢性脂肪神经细胞瘤”应涵盖所有假定的脂肪神经细胞瘤部位。本研究确定了一些形态学、免疫组织化学和遗传学特征,可能有助于脂肪神经细胞瘤的鉴别诊断。此外,手术应是首选治疗方法,完整切除肿瘤应作为目标。需要更多长期随访的病例来制定脂肪神经细胞瘤的最佳治疗方案。

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