Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease: Clinical Course and Management.
Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease: Clinical Course and Management.
复制标题
希佩尔-林道病中的球后血管母细胞瘤：临床过程和治疗。
DOI：
10.1093/neuros/nyaa565
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发表时间：
2021
期刊：
影响因子：
4.8
通讯作者：
Chittiboina,Prashant
中科院分区：
文献类型：
--
作者：
Alvarez,Reinier;Mastorakos,Panagiotis;Hogan,Elizabeth;Scott,Gretchen;Lonser,RussellR;Wiley,HenryE;Chew,EmilyY;Chittiboina,Prashant
背景：在von Hippel-Lindau病（VHL）患者中累及视器的球后血管母细胞瘤是罕见的，文献中仅报道了25例。方法回顾性分析了250例自然史试验中的VHL患者和1774例神经外科治疗方案中的VHL患者的临床病史和影像学资料。回顾所有入选患者的临床过程、磁共振成像、治疗和结局，共有18例球后血管母细胞瘤患者在监测磁共振成像上符合本研究的入选标准。在17例临床信息可用的患者中，10例患者出现与血管母细胞瘤相关的症状，7例无症状。有症状组平均肿瘤体积（810.6±545.5）mm 3大于无症状组（307.6±245.5）mm 3，P<0.05。05）。共有5例有症状的患者接受了手术治疗，所有患者的症状均有所改善。所有3例未接受干预的症状性患者的症状持续进展。对无症状患者的长期连续成像显示，这些肿瘤可以保持放射学稳定和无症状的延长时间（101.43±71个月）。CONCLUSION这项研究表明，球后血管母细胞瘤可能保持稳定和临床无症状的长时间。近期生长和较大的肿瘤体积与症状发生相关。手术治疗有症状的球后血管母细胞瘤是安全的，并可逆转相关症状。
BACKGROUNDRetrobulbar hemangioblastomas involving the optic apparatus in patients with von Hippel-Lindau disease (VHL) are rare, with only 25 reported cases in the literature.OBJECTIVETo analyze the natural history of retrobulbar hemangioblastomas in a large cohort of VHL patients in order to define presentation, progression, and management.METHODSClinical history and imaging of 250 patients with VHL in an ongoing natural history trial and 1774 patients in a neurosurgical protocol were reviewed. The clinical course, magnetic resonance images, treatment, and outcomes were reviewed for all included patients.RESULTSA total of 18 patients with retrobulbar hemangioblastoma on surveillance magnetic resonance imaging met the inclusion criteria for this study. Of the 17 for whom clinical information was available, 10 patients presented with symptoms related to the hemangioblastoma, and 7 were asymptomatic. The mean tumor volume was larger for symptomatic (810.6±545.5 mm 3) compared to asymptomatic patients (307.6±245.5 mm 3; P<. 05). A total of 5 of the symptomatic patients were treated surgically and all experienced improvement in their symptoms. All 3 symptomatic patients that did not undergo intervention had continued symptom progression. Long-term serial imaging on asymptomatic patients showed that these tumors can remain radiographically stable and asymptomatic for extended periods of time (101.43±71 mo).CONCLUSIONThis study suggests that retrobulbar hemangioblastomas may remain stable and clinically asymptomatic for long durations. Recent growth and larger tumor volume were associated with symptom occurrence. Surgical treatment of symptomatic retrobulbar hemangioblastomas can be safe and may reverse the associated symptoms.