外周原始神经外胚叶肿瘤/Ewing's肉瘤的研究进展

外周原始神经外胚叶肿瘤(p PNET,peripheral primitive neuroectodermal tumor)/Ewing's肉瘤(Ewing's Sarcoma,ES)是一类罕见的高度恶性软组织肿瘤,好发于儿童和青年,5年生存率仅20%-30%,高侵袭性生长,易远处转移,易复发,预后不佳。诊断主要依靠病理,手术联合放化疗是主要的治疗方式,分子靶向治疗药物的出现给本病带来了新的希望,但疗效仍需进一步临床资料的验证。目前临床上对本病认识仍不足。本文就p PNET/ES的生物学行为,诊断,治疗和预后的研究进展作一综述,并展望p PNET/ES的研究方向。

Progress of Peripheral Primitive Neuroectodermal Tumor/Ewing's Sarcoma

Peripheral primitive neuroectodermal tumor (pPNET)/ Ewing''s Sarcoma (ES) is a rare malignant soft tissue tumor usually presents in childhood or early adulthood. It is highly invasive and inclined to distant metastasis and relapse, with the overall five-year survival being only 20-30%. The definitive diagnosis of pPNET is based on pathological findings. Surgery combined with radiotherapy and chemotherapy are the primary means of treatment. Molecular targeted drugs have brought new hopes for treating the disease, although the efficacy still needs further confirmation of clinical data. Recent advances on research of the biological behavior, diagnosis, treatment and prognosis of pPNET were summarized in this article. New therapeutic strategies and future research directions were also addressed.