初次出现时具有间变性特征的复发性小脑脂肪神经细胞瘤:一例报告。

IF 0.8 4区 医学 Q4 CLINICAL NEUROLOGY Clinical Neuropathology Pub Date : 2022-03-28 DOI:10.5414/NP301403
M. Maharaj, S. Tawfik, F. Ziad, T. Muthu
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引用次数: 0

摘要

背景小脑脂肪神经细胞瘤是一种罕见的实体瘤,迄今为止,在现有文献中报告的病例和系列不到100例。尽管小脑仍然是典型的原发部位,但该实体已被证明具有增强的侵袭性和恶性进展,并伴有多次复发。病例描述我们报告了一个独特的病例,一位64岁的绅士患有小脑脂肪神经细胞瘤,伴有多次复发和进行性发育不良。肿瘤在第一次出现时表现出间变性特征,尽管进行了手术切除和术后放疗,但在短短2年内以更具侵袭性的方式复发。它在6个月内再次复发,随后在没有进一步放疗的情况下再次揭穿。在对患者第二次复发进行手术治疗近3年后的最新随访中,患者保持良好,神经损伤最小,没有复发的放射学迹象。结论小脑脂肪神经细胞瘤可能表现出越来越不典型的组织学特征,这可能需要更积极的术后治疗来防止疾病复发和临床恶化。这可能包括更积极的手术切除范围,并在所有病例中考虑辅助放射治疗。
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Recurrent cerebellar liponeurocytoma with anaplastic features at initial presentation: A case report.
BACKGROUND Cerebellar liponeurocytoma is a rare entity with fewer than 100 reported cases and series in the available literature to date. Although the cerebellum remains the typical primary site, the entity has been shown to demonstrate increased aggressiveness and malignant progression with multiple recurrences. CASE DESCRIPTION We present a unique case in a 64-year-old gentleman of a cerebellar liponeurocytoma with multiple recurrences and progressive anaplasia. The tumor showed anaplastic features at first presentation and recurred in a more aggressive fashion in a short 2-year period despite surgical debulking and post-operative radiotherapy. It re-recurred within 6 months with subsequent re-debulking without further radiotherapy. At latest follow-up almost 3 years since surgical management of the patient's second recurrence, the patient remains well with minimal neurological impairment and no radiological signs of recurrence. CONCLUSION Cerebellar liponeurocytoma may present with increasingly atypical histological features that may warrant more aggressive post-operative treatment to prevent disease recurrence and clinical deterioration. This may include a more aggressive surgical resection margin and consideration of adjuvant radiotherapy in all cases.
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来源期刊
Clinical Neuropathology
Clinical Neuropathology 医学-病理学
CiteScore
1.60
自引率
0.00%
发文量
70
审稿时长
>12 weeks
期刊介绍: Clinical Neuropathology appears bi-monthly and publishes reviews and editorials, original papers, short communications and reports on recent advances in the entire field of clinical neuropathology. Papers on experimental neuropathologic subjects are accepted if they bear a close relationship to human diseases. Correspondence (letters to the editors) and current information including book announcements will also be published.
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