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突变基因 综合征 占所有PCC/PGL
比例(%)年龄
(岁)PCC比例
(%)PGL比例
[%(交感/副交感)]恶性比例
(%)RET MEN2 5.3 35.6(4~73) 100 0 2.9 VHL VHL 9.0 28.6(5~67) 90.3 18.6(5.9/ 8.8) 3.4 NF1 NF1 2.9 41.6(1~74) 95.3 6.1(6.1/ 0) 9.3 SDHD PGL1 7.1 35.0(10~96) 23.9 91.5(22.0/84.4) 3.5 SDHAF2 PGL2 0 32.2(20~59) 0 100 (0 /100) 0 SDHC PGL3 0.5 42.7(13~73) 0 100 (7.1/92.9) 0 SDHB PGL4 5.5 32.7(6~77) 25.2 77.5(70.7/24.4) 30.7 SDHA HNPGL < 3 40.0(27~55) 16.7 83.3(50.0/33.3) 0~14.3 KIF1Bβ - 0 46.0(22~70) 100 0 0 EGLN1 - 0 43.0 0 100 (100 / 0) 0 TMEM127 - < 2 42.8(21~72) 95.7 8.7(4.3/ 4.3) 4.3 MAX - NA 32.2(17~47) 100 0 25.0 SDHB, C, D Carney-Stratakis综合征 0 33.0(10~61) 9.1 100 0 MEN1 MEN1 0 30.5(29~32) 100 0 14.3 未突变 散发性 70 48.3(5~93) 72.9 29.1(8.8/20.3) 8.9 PCC:嗜铬细胞瘤;PGL:副神经节瘤;NA:未见报道 -
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