Arterial Spin-Labeling Perfusion Lightbulb Sign: An Imaging Biomarker of Pediatric Posterior Fossa Hemangioblastoma.

Onur Simsek, Nakul Sheth, Amirreza Manteghinejad, Mix Wannasarnmetha, Timothy P Roberts, Aashim Bhatia
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Abstract

Background and purpose: Hemangioblastoma is a rare vascular tumor that occurs within the central nervous system in children. Differentiating hemangioblastoma from other posterior fossa tumors can be challenging on imaging, and preoperative diagnosis can change the neurosurgical approach. We hypothesize that a "lightbulb sign" on the arterial spin-labeling (ASL) sequence (diffuse homogeneous intense hyperperfusion within the solid component of the tumor) will provide additional imaging finding to differentiate hemangioblastoma from other posterior fossa tumors.

Materials and methods: In this retrospective comparative observational study, we only included pathology-proved cases of hemangioblastoma, while the control group consisted of other randomly selected pathology-proved posterior fossa tumors from January 2022 to January 2024. Two blinded neuroradiologists analyzed all applicable MRI sequences, including ASL sequence if available. ASL was analyzed for the lightbulb sign. Disagreements between the radiologists were resolved by a third pediatric neuroradiologist. χ2 and Fisher exact test were used to analyze the data.

Results: Ninety-five patients were enrolled in the study; 57 (60%) were boys. The median age at diagnosis was 8 years old (interquartile range: 3-14). Of the enrolled patients, 8 had hemangioblastoma, and 87 had other posterior fossa tumors, including medulloblastoma (n = 31), pilocytic astrocytoma (n = 23), posterior fossa ependymoma type A (n = 16), and other tumors (n = 17). The comparison of hemangioblastoma versus nonhemangioblastoma showed that peripheral edema (P = .02) and T2-flow void (P = .02) favor hemangioblastoma, whereas reduced diffusion (low ADC) (P = .002) and ventricular system extension (P = .001) favor nonhemangioblastoma tumors. Forty-two cases also had ASL perfusion sequences. While high perfusion favors hemangioblastoma (P = .03), the lightbulb sign shows a complete distinction because all the ASL series of hemangioblastoma cases (n = 4) showed the lightbulb sign, whereas none of the nonhemangioblastoma cases (n = 38) showed the sign (P < .001).

Conclusions: Lightbulb-like intense and homogeneous hyperperfusion patterns on ASL are helpful in diagnosing posterior fossa hemangioblastoma in children.

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ASL 灌注灯泡信号:小儿后窝血管母细胞瘤的成像生物标志物。
背景和目的:血管母细胞瘤是一种发生在儿童中枢神经系统内的罕见血管肿瘤。在影像学上将血管母细胞瘤与其他后窝肿瘤区分开来具有挑战性,术前诊断可改变神经外科手术方法。我们假设 ASL 序列上的 "灯泡征"(肿瘤实性成分内弥漫均匀的高灌注)将为区分血管母细胞瘤和其他后窝肿瘤提供额外的影像学发现:在这项回顾性对比观察研究中,我们只纳入了经病理学证实的血管母细胞瘤病例,而对照组则由 2022 年 1 月至 2024 年 1 月期间随机选取的其他经病理学证实的后窝肿瘤组成。两名双盲神经放射学专家分析了所有适用的 MRI 序列,包括 ASL 序列(如有)。对 ASL 进行了 "灯泡征 "分析。放射科医生之间的分歧由第三位儿科神经放射科医生解决。数据分析采用卡方检验和费雪精确检验:95名患者参与了研究,其中57人(60%)为男性。确诊时的中位年龄为 8 岁(IQR:3-14)。在登记的患者中,8人患有血管母细胞瘤,87人患有其他后窝肿瘤,包括髓母细胞瘤(31人)、朝粒细胞星形细胞瘤(23人)、后窝上皮瘤A型(16人)和其他肿瘤(17人)。血管母细胞瘤与非血管母细胞瘤的比较显示,周围水肿(p=0.02)和T2血流空洞(p=0.02)有利于血管母细胞瘤,而弥散减少(低ADC)(p=0.002)和脑室系统扩展(p=0.001)有利于非血管母细胞瘤。虽然高灌注有利于血管母细胞瘤(p=0.03),但灯泡征显示出完全的区别,因为所有血管母细胞瘤病例(n=4)的 ASL 序列都显示出灯泡征,而非血管母细胞瘤病例(n=38)则无一显示出灯泡征(p结论:ASL=动脉自旋标记;pASL=脉冲动脉自旋标记;pCASL=假连续动脉自旋标记;DCE=动态对比增强;DSC=动态感性对比;VHL=冯-希佩尔-林道。
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