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1型成人神经纤维瘤病患者中的非视神经胶质瘤样病变

Non-Optic Glioma-like Lesions in Adult Neurofibromatosis Type 1 Patients.

作者信息

Taal Walter, Zick Bart, Emmer Bart J, van den Bent Martin J

机构信息

Department of Neuro-Oncology/Neurology, Erasmus MC Cancer Institute, 3015 GD Rotterdam, The Netherlands.

Department of Radiology, Erasmus MC, Dr. Molewaterplein 40, 3015 GD Rotterdam, The Netherlands.

出版信息

Diagnostics (Basel). 2024 Dec 30;15(1):67. doi: 10.3390/diagnostics15010067.

Abstract

Physicians face clinical dilemmas in the diagnosis of non-optic intraparenchymal lesions on MRI brain scans of patients with neurofibromatosis type 1. As the incidence and evolution of these lesions into adulthood remain unclear, we conducted a retrospective study on this topic. All adult neurofibromatosis type 1 patients who had at least one MRI brain scan in our center were selected for this study. Brain lesions with contrast enhancement after gadolinium administration and/or mass effect were named "glioma-like lesions". In our cohort of 396 adult neurofibromatosis type 1 patients, 182 had at least one MRI scan of the brain. A total of 48 glioma-like lesions were found in 38/182 patients. The majority of glioma-like lesions remained stable, decreased in size or even disappeared during a median follow-up time of 8.5 years. Twelve glioma-like lesions in 11/182 patients were resected or biopsied, and histology showed gliomas of astrocytic origin (WHO grade 1-4). It was concluded from these data that asymptomatic glioma-like lesions on MRI brain scans in neurofibromatosis type 1 patients, either with contrast enhancement and/or mass effect, had an indolent nature. Mildly symptomatic or asymptomatic patients can therefore be followed without invasive diagnostic and therapeutic procedures.

摘要

在对1型神经纤维瘤病患者进行脑部MRI扫描时,医生在诊断非视神经实质内病变方面面临临床困境。由于这些病变在成年期的发病率和演变情况尚不清楚,我们针对这一主题进行了一项回顾性研究。本研究选取了在我们中心至少进行过一次脑部MRI扫描的所有成年1型神经纤维瘤病患者。钆剂注射后有强化和/或占位效应的脑部病变被称为“胶质瘤样病变”。在我们的396例成年1型神经纤维瘤病患者队列中,182例至少进行过一次脑部MRI扫描。在38/182例患者中总共发现了48个胶质瘤样病变。在中位随访时间8.5年期间,大多数胶质瘤样病变保持稳定、缩小甚至消失。182例患者中的11例有12个胶质瘤样病变接受了切除或活检,组织学显示为星形细胞起源的胶质瘤(世界卫生组织1 - 4级)。从这些数据得出的结论是,1型神经纤维瘤病患者脑部MRI扫描上无症状的胶质瘤样病变,无论有无强化和/或占位效应,都具有惰性。因此,轻度症状或无症状的患者可以在不进行侵入性诊断和治疗程序的情况下进行随访。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3202/11720024/83b484e11f3d/diagnostics-15-00067-g002.jpg

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