Teranishi Yu, Hongou Hiroki, Miyawaki Satoru, Saito Nobuhito
Department of Neurosurgery, the University of Tokyo.
No Shinkei Geka. 2022 Jan;50(1):141-149. doi: 10.11477/mf.1436204539.
Solitary fibrous tumor(SFT)/hemangiopericytoma(HPC)is a rare mesenchymal tumor with propensity for recurrence and metastasis. Although SFT and HPC were initially considered to be distinct entities, the identification of NAB2-STAT6 fusion as a definitive molecular alteration in both tumors has led to their integration into one disease entity, for both meningeal and non-meningeal lesions. This fusion leads to a nuclear relocation of the STAT6 protein and is detectable with immunohistochemistry. STAT6 immunohistochemistry has been shown to have excellent sensitivity and specificity for histological diagnosis. Although these discoveries have improved the diagnosis of SFT/HPC, the association of the NAB2-STAT6 fusion status with phenotype and prognosis remains unclear, and accurate prognostic factors have not been established. This comprehensive review provides current knowledge on the clinical, histological, and molecular characteristics of SFT/HPC.
孤立性纤维性肿瘤(SFT)/血管外皮细胞瘤(HPC)是一种罕见的间叶性肿瘤,有复发和转移倾向。尽管SFT和HPC最初被认为是不同的实体,但NAB2-STAT6融合作为这两种肿瘤中明确的分子改变被发现后,无论是脑膜还是非脑膜病变,它们都被归为一种疾病实体。这种融合导致STAT6蛋白核易位,可通过免疫组织化学检测到。STAT6免疫组织化学已被证明对组织学诊断具有出色的敏感性和特异性。尽管这些发现改善了SFT/HPC的诊断,但NAB2-STAT6融合状态与表型和预后的关系仍不清楚,准确的预后因素尚未确立。这篇综述提供了关于SFT/HPC临床、组织学和分子特征的当前知识。