原始神经外胚层肿瘤的研究进展

Research progress of primitive neuroectodermal tumor

原始神经外胚层肿瘤(PNET)是罕见的高度恶性小圆细胞肿瘤,可分为中枢型(cPNET)与外周型(pPNET)。两者除发病部位不同以外,在好发年龄、转移途径、免疫组化染色、染色体易位等方面也不同。因此需对两者进行鉴别,尤其是病灶邻近脑膜或位于椎管内时更有必要。对cPNET及pPNET的肿瘤遗传学、临床表现、病理诊断、影像表现、治疗和预后,以及cPNET与pPNET,cPENT与髓母细胞瘤,pPNET与尤文氏肉瘤之间的关系等进行综述。

Primitive neuroectodermal tumor is a rare and highly malignant small round cell tumor. It can be divided into central PNET （cPNET） and peripheral PNET （pPNET）. In addition to the lesions’ location, however,they are different in predilection age, route of metastasis, immunohistochemical staining, and chromosome translocation. So it is very necessary to identify each other, especially, when the lesions are close to the meninges or located in the vertebral canal. The aim of this review was to investigate the tumor genetics, clinical manifestation, pathologic diagnosis, imaging findings, treatment, and prognosis of the two kinds of tumors, also investigate the relationships between cPNET and pPNET, cPENT and medulloblastoma, pPNET and Ewing’s sarcoma.