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成人起病的髓母细胞瘤表现为生长缓慢的非典型肿块:一例报告

Adult-onset medulloblastoma presenting as slow-growing, atypical mass: a case report.

作者信息

van der Kolk Anja Gwendolyn, van Leeuwen Roeland B, Poulsen Louise

机构信息

Department of Neurology, Gelre Ziekenhuizen, Apeldoorn, Netherlands.

Department of Radiology, Gelre Ziekenhuizen, Apeldoorn, Netherlands.

出版信息

BJR Case Rep. 2017 Jan 25;3(2):20160115. doi: 10.1259/bjrcr.20160115. eCollection 2017.

Abstract

Medulloblastoma accounts for < 1% of all primary central nervous system tumours in adults. Although a "classical" imaging presentation -being a well-defined mass, often located in the cerebellar hemisphere, with surrounding oedema, showing iso- and heterogeneous signal intensity and contrast enhancement -has been described, case reports and series have also shown the extremely heterogeneous imaging aspect of this tumour , reflecting its heterogeneous molecular phenotype. Owing to the general location of the tumour within the cerebellopontine angle, causing (fast) progressive symptoms of headache and gait instability, most patients present within 3  months from symptom onset. This case report describes a presentation of adult medulloblastoma not shown before, with an indolent course over a period of 4.5  years, initially without clear abnormalities on imaging. It highlights the importance of including medulloblastoma in the differential diagnosis of all lesions found near/continuous with the fourth ventricle in the adult population, even when clinical onset and imaging characteristics do not suggest "classical" medulloblastoma.

摘要

髓母细胞瘤在成人原发性中枢神经系统肿瘤中占比不到1%。尽管已经描述了一种“典型”的影像学表现——为边界清晰的肿块,通常位于小脑半球,伴有周围水肿,呈等信号和不均匀信号强度以及强化——但病例报告和系列研究也显示出该肿瘤极其多样的影像学表现,反映了其异质性分子表型。由于肿瘤一般位于桥小脑角内,导致(快速)进展性头痛和步态不稳症状,大多数患者在症状出现后3个月内就诊。本病例报告描述了一种此前未见过的成人髓母细胞瘤表现,病程长达4.5年,进展缓慢,最初影像学检查无明显异常。它强调了在成人中,即使临床起病和影像学特征不提示“典型”髓母细胞瘤,在鉴别诊断所有位于第四脑室附近/与第四脑室连续的病变时,将髓母细胞瘤纳入鉴别诊断的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7333/6159246/b0a3d33f8965/bjrcr.20160115.g001.jpg

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