Department of Neurosurgery, Beaumont Hospital, Dublin, Ireland.
Department of Neuroscience, Royal College of Surgeons in Ireland, Dublin, Ireland.
CNS Oncol. 2020 Dec 1;9(4):CNS66. doi: 10.2217/cns-2020-0021. Epub 2020 Nov 27.
We report the third presentation of an intermixed arteriovenous malformation and hemangioblastoma. The rare occurrence of the diagnostic histologic features of both a neoplasm and vascular malformation in a single lesion is more common in gliomas, as angioglioma, and is termed an 'intermixed' lesion. We review the literature concerning the developmental biology of each lesion, and potential interplay in the formation of an intermixed vascular neoplasm and vascular malformation. The roles of cellular origin, genetic susceptibility, favourable microenvironment, altered local gene expression and key regulatory pathways are reviewed. Our review supports angiography and genetic profiling in intermixed lesions to inform management strategies. Consideration should be given to multimodality therapeutic interventions as required, including microsurgical resection, stereotactic radiosurgery and further research to exploit emerging molecular targets.
我们报告了第三例动静脉畸形和血管母细胞瘤的混合表现。在单个病变中同时存在肿瘤和血管畸形的罕见诊断组织学特征在神经胶质瘤中更为常见，如血管胶质瘤，并被称为“混合”病变。我们回顾了关于每种病变的发育生物学以及混合性血管肿瘤和血管畸形形成中的潜在相互作用的文献。我们回顾了细胞起源、遗传易感性、有利的微环境、局部基因表达改变和关键调节途径的作用。我们的综述支持在混合病变中进行血管造影和基因分析，以指导治疗策略。应根据需要考虑多模式治疗干预，包括显微外科切除、立体定向放射外科和进一步研究以利用新兴的分子靶点。
