副神经节瘤是一种神经内分泌肿瘤,根据部位分为肾上腺外副神经节瘤和肾上腺内副神经节瘤(即嗜铬细胞瘤),2 者合称为副神经节瘤和嗜铬细胞瘤(pheochromocytoma and paraganglioma,PPGL).随着对PPGL分子生物学的研究不断深入,目前认为PPGL可分为假缺氧驱动型、激酶信号通路改变型和Wnt通路改变型3个亚型,对于不同亚型PPGL给予不同的个体化治疗.本文对PPGL不同分子分型及其个体化治疗的最新进展进行综述.
Research progress in molecular typing and individualized therapy of paraganglioma
Paraganglioma(PGL)is a neuroendocrine tumor,which can be divided into extradrenal paraganglioma and intradrenal paraganglioma according to its location.Together they are called PPGL(pheochromocytoma and paraganglioma).With the further study of its molecular biology,PPGL can be divided into three subtypes:pseudohypoxia-driven type,kinase pathway altered type and Wnt pathway altered type.Different subtypes of PPGL can provide different individualized treatments.In this paper,we review the recent advances in different molecular typing and individualized therapy of PPGL.
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