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小脑脂肪神经细胞瘤——一种罕见疾病:病例报告

Cerebellar liponeurocytoma - a rare entity: a case report.

作者信息

Gembruch Oliver, Junker Andreas, Ahmadipour Yahya, Sure Ulrich, Lemonas Elias

机构信息

Department of Neurosurgery, University Hospital Essen, University of Duisburg-Essen, Hufelandstrasse 55, 45122, Essen, Germany.

Department of Neuropathology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.

出版信息

J Med Case Rep. 2018 Jun 17;12(1):170. doi: 10.1186/s13256-018-1706-z.

DOI:10.1186/s13256-018-1706-z
PMID:29908563
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6004288/
Abstract

BACKGROUND

Cerebellar liponeurocytoma is a rare tumor of the central nervous system occurring mainly in the posterior fossa, which shows neuronal and variable astrocytic differentiation with foci of lipomatous differentiation. Liponeurocytoma develops in adult patients and is defined in the World Health Organization classification of 2016 as a rare benign grade II tumor.

CASE PRESENTATION

A 39-year-old Italian man presented to our department suffering from headache and nausea. Magnetic resonance imaging revealed a right-sided cerebellar lesion showing poor contrast enhancement without an obstructive hydrocephalus. Surgery was indicated and total tumor resection was achieved. He was discharged without any neurological deficits. Histopathological examinations revealed a cerebellar liponeurocytoma. A neurological follow-up examination revealed no neurological deficit directly after surgery and 1 year later. Radiotherapy was recommended at the neurooncological board despite the total removal of the tumor, but our patient refused adjuvant radiotherapy. Magnetic resonance imaging of his neurocranium with and without contrast enhancement 48 hours after surgery and 15 months after surgery showed no residual tumor.

CONCLUSIONS

Liponeurocytomas are rare benign tumors occurring in the majority of cases in the cerebellum. The therapy of choice is surgery. Postoperative radiotherapy has to be discussed individually, but seems to be sufficient if complete tumor resection is not achieved or in cases of a tumor recurrence.

摘要

背景

小脑脂肪神经细胞瘤是一种罕见的中枢神经系统肿瘤,主要发生于后颅窝,具有神经元及不同程度的星形细胞分化,并伴有脂肪化生灶。脂肪神经细胞瘤多见于成年患者,在2016年世界卫生组织分类中被定义为一种罕见的良性II级肿瘤。

病例介绍

一名39岁的意大利男子因头痛和恶心前来我院就诊。磁共振成像显示右侧小脑病变,增强扫描显示强化不佳,无梗阻性脑积水。遂行手术治疗,肿瘤全切。患者出院时无任何神经功能缺损。组织病理学检查显示为小脑脂肪神经细胞瘤。术后及术后1年的神经功能随访检查均未发现神经功能缺损。尽管肿瘤已全切,但神经肿瘤学委员会仍建议行放疗,但患者拒绝辅助放疗。术后48小时及术后15个月的头颅磁共振成像增强扫描及平扫均未发现残留肿瘤。

结论

脂肪神经细胞瘤是一种罕见的良性肿瘤,多数发生于小脑。首选治疗方法是手术。术后放疗需个体化讨论,但如果肿瘤未全切或复发,放疗似乎是必要的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67fb/6004288/56fff98fc3b6/13256_2018_1706_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67fb/6004288/5423201c54ba/13256_2018_1706_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67fb/6004288/26ff8c1f3a1b/13256_2018_1706_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67fb/6004288/56fff98fc3b6/13256_2018_1706_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67fb/6004288/5423201c54ba/13256_2018_1706_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67fb/6004288/26ff8c1f3a1b/13256_2018_1706_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67fb/6004288/56fff98fc3b6/13256_2018_1706_Fig3_HTML.jpg

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