率5%106,1。但长期疗效欠佳，2年内几平均有复发或转移。主要副作用是骨髓抑制。核素 标记的奥曲肽可用于MBG阴性者，但疗效尚难评价。 3.放疗和化疗外放射治疗推荐于无法手术切除的肿痘和缓解骨转移所致疼痛，但可 能加重高血压101。化疗推荐CVD方案（环磷酰胶、长春新碱、氯烯唑胺），有效率约50%， 但多于2年内复发9,1H0。联合MBG可能提高疗效1。抗血管生成靶向药物治疗可能有效 02,142-116。 4.处理儿茶酚胺增多症对于恶性或因故不能手术者推荐α-受体阻滞剂、B受体阻滞剂 控制高血压。 六、预后和随访 (一)预后 PHEO/PGL的预后与年龄、良恶性、有无家族史及治疗早晚等有关。良性者5年生存率 >95%,但约50%志者仍持续高血压。复发率为6.5%一17%，复发者恶性率约50%，家族 性、肾上腺外及右侧者更易复发。恶性PHE0/PGL不可治愈，5年生存率约50%1叫，肝、 肺转移较骨转移者预后差46,1021，其中约50%死于1~3年，但约50%可存活20年以上4。 (二)随访 1.随访原因 (1)肿瘤有无残留： (2)病理难于鉴别良恶性，主要依据其临床出现转移： (3)易复发、多发，特别是家族发病者。 2.随访内容：包括临床症状（如高血压）、生化指标（如血浆游离MNS、24h尿CA和 分馏的MNS)、CT扫描等。 3.随访方案 (1)推荐术后10~14天复查血尿生化指标6,11，判断肿瘤是否残留、有无转移等。 (2)敢发病例单侧肾上腺切除者每年一次，至少连续10年1刀。 (3)高危群体(SDHB突变、PGL、肿瘤体积巨大)和遗传性PHEO/PGL者每6~12个月 复查1次临床和生化指标，终生随访1
16 率5%[106, 107]。但长期疗效欠佳，2年内几乎均有复发或转移。主要副作用是骨髓抑制。核素 标记的奥曲肽可用于MIBG阴性者，但疗效尚难评价。 3．放疗和化疗 外放射治疗推荐于无法手术切除的肿瘤和缓解骨转移所致疼痛，但可 能加重高血压[108]。化疗推荐 CVD 方案（环磷酰胺、长春新碱、氮烯唑胺），有效率约50%， 但多于２年内复发[109, 110]。联合MIBG可能提高疗效[111]。抗血管生成靶向药物治疗可能有效 [102, 112-116]。 4. 处理儿茶酚胺增多症 对于恶性或因故不能手术者推荐α-受体阻滞剂、β-受体阻滞剂 控制高血压[8]。 六、预后和随访 （一）预后 PHEO/PGL的预后与年龄、良恶性、有无家族史及治疗早晚等有关。良性者5年生存率 ＞95%，但约50%患者仍持续高血压[117]。复发率为6.5%～17%，复发者恶性率约50%，家族 性、肾上腺外及右侧者更易复发[6]。恶性PHEO/PGL不可治愈，5年生存率约 50%[102]，肝、 肺转移较骨转移者预后差[46, 102]，其中约50%死于1～3年，但约50%可存活20年以上[84]。 （二）随访 1．随访原因 （1）肿瘤有无残留； （2）病理难于鉴别良恶性，主要依据其临床出现转移； （3）易复发、多发，特别是家族发病者。 2．随访内容：包括临床症状（如高血压）、生化指标（如血浆游离MNs、24h尿CA和 分馏的MNs）、CT扫描等。 3．随访方案 （1）推荐术后10～14天复查血尿生化指标[36, 118]，判断肿瘤是否残留、有无转移等。 （2）散发病例单侧肾上腺切除者每年一次，至少连续10年[117]。 （3）高危群体（SDHB突变、PGL、肿瘤体积巨大）和遗传性PHEO/PGL者每6～12个月 复查1次临床和生化指标，终生随访[119]
七、PHE0/PGL诊断流程图29,30,120,12 临床症状可疑如 ·发作性或顾固性高血压 。麻醉、手术、妊娠中血压剧烈波动 肾上腺或腹主动脉旁偶发肿物 PHEO/PGL家族史 生化检测≥2次 。血浆游离MN、NMN ·24h尿MN、NMN ·24h尿CA 升高>2倍 临界 正常 生化证实 重复生化检查、—④症状依旧或高危 质品 症状依旧一⊙一→排除PHEO1PGL ⊕ 6月后重复生化检查 无肿物肿物 影像功能定位 ·MIBG PET 胸部及头颈CT/MRI →肿物 ·奥曲肽显像 ⊙ 无肿物 MIBG/奥曲肽/PET PHEO/PGL 基因筛查（可选） 排除PHEO1PGL
17 七、PHEO/PGL诊断流程图[29, 30, 120, 121] 无肿物 肿 物 胸部及头颈 CT / MRI 无肿物 肿 物 排除 PHEO / PGL MIBG/奥曲肽/PET - + 影像功能定位 ● MIBG ● 奥曲肽显像 + PHEO / PGL 基因筛查(可选) + - PET - 重复生 化检查 临床症状可疑如： ● 发作性或顽固性高血压 ● 麻醉、手术、妊娠中血压剧烈波动 肾上腺或腹主动脉旁偶发肿物 PHEO/PGL 家族史 生化检测≥2 次 ● 血浆游离 MN、NMN ● 24h 尿 MN、NMN ● 24h 尿 CA 升高＞2 倍 临 界 正 常 生化证实 + 重复生化检查 + 症状依旧或高危 - - 影 像 解 剖 定 位 （腹部+盆腔 CT / MRI） 6 月后重复生化检查 症状依旧 - 排除 PHEO / PGL +
八、PHE0PGL治疗流程图29,0,110,12四 嗜铬细胞痴/副神经节瘤(PHEO/PGL） 药物准备 ·酚苄明 。哌唑嗪、多沙唑嗪 ·钙离子洁抗剂 。高血压危象：硝普钠、乌拉地尔 ·心律失常：B.受体阻滞剂 性 肾上腺 上腺外 原发局限病灶 转移病灶 能否“根治”切除？ 可切除 不可切除 散发性遗传性 原发灶+ 移灶切除 肿瘤切除 ·3-MIBG ·化 131-MIBG 。放疗 随访≥10年 终生随访 (a)MIBG昆像阳性者 (b)奥曲肽显像阳性者
18 八、PHEO/PGL治疗流程图[29, 30, 110, 122] 嗜铬细胞瘤/副神经节瘤（PHEO/PGL） 药物准备 ● 酚苄明 ● 哌唑嗪、多沙唑嗪 ● 钙离子拮抗剂 ● 高血压危象：硝普钠、乌拉地尔 ● 心律失常：β-受体阻滞剂 良 性 恶 性 肾上腺 肾上腺外 原发局限病灶 转移病灶 单 侧 双 侧 能否“根治”切除？ + - 可切除 不可切除 散发性 遗传性 原发灶+转 移灶切除 减瘤 手术 肿瘤切除 保留肾上腺 的肿瘤切除 肿瘤 切除 “根治” 切除 减瘤 切除 ● 131I-MIBG (a) ● 奥曲肽 (b) ● 化疗 131I-MIBG (a) ● 放疗 随访≥10 年 终生随访 （a）MIBG 显像阳性者 （b）奥曲肽显像阳性者
参考文献 M. elli M.et a Phaeochro 28 26 es.-ic et The 2 eoeof neromast peepe 阁 阁 prospects.Endocr Rev. Agarwal SC,et al Phacochro ytomas presenting as acute rises afer beta 【18 E.Da adNExra-adrenal Nephrol,( f unusual locations of extra-adrenal 05.1s8592 22 23 24 19.0 H Endocrinol Diabetes 26 291 2331-9 Karag iann B phe 潘东亮，李汉忠，曾正略，咯铬细胞瘤临床功能分级与术前准名标准的深讨：中华外科杂志 M m 200). omocytoma. B
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