Alsufayan Reema, Alcaide-Leon Paula, de Tilly Lyne Noel, Mandell Daniel M, Krings Timo
University of Toronto, Toronto, Canada.
University of Toronto, St. Michael's Hospital, Toronto, Canada.
Neuroradiology. 2017 Sep;59(9):873-883. doi: 10.1007/s00234-017-1884-1. Epub 2017 Jul 27.
Multinodular and vacuolating neuronal tumor (MVNT) have been recently added to the WHO classification of CNS tumors and has not been extensively reported upon in the radiological literature. We report the first radiological and the largest series of cases, aiming to highlight the natural history of lesions with the imaging appearance of MVNT with long follow-up time.
In this retrospective study, we collected cases with the imaging appearance of MVNT. All lesions were evaluated by using routine MR imaging, with follow-up of up to 93 months. Patient demographics, clinical course, and MRI features of the lesions were recorded.
Twenty-four subjects were enrolled, f/m = 16:8, age range 24-59 years, with a median age of 45 years. The patients' symptoms were often episodic and most frequently due to headaches in 12 (50%), visual symptoms in 6 (25%), seizures in 5 ± 1 (20-25%), paresthesia in 4 (17%), cognitive difficulties in 4 (17%), in addition to other variable neurological symptoms, or incidental. A total of 30 lesions identified, 77% of the lesions had gadolinium-enhanced MRI and only 13% showed enhancement. A 6.7% of the lesions that had MRI followed up showed progression, while the rest remained stable up to 93 months interval. All patients had intact neurological examinations (except one case that was diagnosed with optic neuritis), were managed conservatively, and did well.
The natural history of lesions with imaging features of MVNT is overall stable from a clinical and imaging appearance over time.
多结节空泡状神经元肿瘤(MVNT)最近被纳入世界卫生组织中枢神经系统肿瘤分类,但在放射学文献中尚未有广泛报道。我们报告首例放射学病例及最大系列病例,旨在通过长期随访突出具有MVNT影像表现的病变的自然病程。
在这项回顾性研究中,我们收集了具有MVNT影像表现的病例。所有病变均采用常规磁共振成像进行评估,随访时间长达93个月。记录患者的人口统计学资料、临床病程及病变的MRI特征。
纳入24例受试者,男女比例为16:8,年龄范围24 - 59岁,中位年龄45岁。患者症状常呈发作性,最常见的是头痛(12例,50%)、视觉症状(6例,25%)、癫痫发作(5±1例,20 - 25%)、感觉异常(4例,约17%)、认知困难(4例,约17%),此外还有其他各种神经系统症状或为偶然发现。共识别出30个病变,77%的病变在MRI上有钆增强表现,仅13%有强化。在接受MRI随访的病变中,6.7%有进展,其余在长达93个月的间隔期内保持稳定。所有患者神经检查均正常(除1例诊断为视神经炎),采用保守治疗,情况良好。
具有MVNT影像特征的病变的自然病程在临床和影像表现上总体随时间推移保持稳定。