毛细胞黏液样星形细胞瘤的研究进展

毛细胞黏液样星形细胞瘤（pilomyxoid astrocytoma,PMA）是2007年WHO中枢神经系统肿瘤病理学中新提出的一个分类。它是毛细胞星形细胞瘤（pilocytic astrocytoma,PA）的特殊亚型,患者发病年龄更早,视交叉/下丘脑是病变的常见部位。近些年也有成人PMA及特殊部位PMA的报道。组织学见双极梭形细胞围绕血管生长,有黏液样背景,无PA常见的Rosenthal纤维和嗜酸性颗粒小体。与PA相比,患者预后较差,易局部多次复发或经脑脊液转移甚至死亡。PMA复发后可演变呈典型的PA。

Pilomyxoid astrocytoma:literature review

Pilomyxoid astrocytoma (PMA) is a new variant of pilocytic astrocytoma (PA) in the WHO classification of central nervous system tumor in 2007. The patients of PMA are younger. Optic chiasm/hypothalamus is the common location of it. Recently, there were some reports of PMA in adult or in special locations. The spindle cells grow around the vessels with the mucus-like background and are lack of typical Rosenthal fibers or eosinophilic granular bodies. Compared with PA, PMA is usually local recurrent and of metastasis by cerebrospinal fluid which may cause death. PMA can translate to typical PA after local recurrence.