Fitzgerald Paul A, Goldsby Robert E, Huberty John P, Price David C, Hawkins Randall A, Veatch Janet J, Dela Cruz Filemon, Jahan Thierry M, Linker Charles A, Damon Lloyd, Matthay Katherine K
Department of Medicine, UCSF Comprehensive Cancer Center, Box 1222, University of California, San Francisco, San Francisco, CA 94143-1222, USA.
Ann N Y Acad Sci. 2006 Aug;1073:465-90. doi: 10.1196/annals.1353.050.
Thirty patients with malignant pheochromocytoma (PHEO) or paraganglioma (PGL) were treated with high-dose 131I-MIBG. Patients were 11-62 (mean 39) years old: 19 patients males and 11 females. Nineteen patients had PGL, three of which were multifocal. Six PGLs were nonsecretory. Eleven patients had PHEO. All 30 patients had prior surgery. Fourteen patients were refractory to prior radiation or chemotherapy before 131I-MIBG. Peripheral blood stem cells (PBSCs) were collected and cryopreserved. 131I-MIBG was synthesized on-site, by exchange-labeling 131I with 127I-MIBG in a solid-phase Cu2+-catalyzed exchange reaction. 131I-MIBG was infused over 2 h via a peripheral IV. Doses ranged from 557 mCi to 1185 mCi (7.4 mCi/kg to 18.75 mCi/kg). Median dose was 833 mCi (12.55 mCi/kg). Marrow hypoplasia commenced 3 weeks after 131I-MIBG therapy. After the first 131I-MIBG therapy, 19 patients required platelet transfusions; 19 received GCSF; 12 received epoeitin or RBCs. Four patients received a PBSC infusion. High-dose 131I-MIBG resulted in the following overall tumor responses in 30 patients: 4 sustained complete remissions (CRs); 15 sustained partial remissions (PRs); 1 sustained stable disease (SD); 5 progressive disease (PD); 5 initial PRs or SD but relapsed to PD. Twenty-three of the 30 patients remain alive; deaths were from PD (5), myelodysplasia (1), and unrelated cause (1). Overall predicted survival at 5 years is 75% (Kaplan Meier estimate). For patients with metastatic PHEO or PGL, who have good *I-MIBG uptake on diagnostic scanning, high-dose 131I-MIBG therapy was effective in producing a sustained CR, PR, or SD in 67% of patients, with tolerable toxicity.
30例恶性嗜铬细胞瘤（PHEO）或副神经节瘤（PGL）患者接受了大剂量131I-间碘苄胍（MIBG）治疗。患者年龄为11至62岁（平均39岁）：男性19例，女性11例。19例患者患有PGL，其中3例为多灶性。6例PGL无分泌功能。11例患者患有PHEO。所有30例患者均曾接受过手术。14例患者在接受131I-MIBG治疗前对先前的放疗或化疗无效。采集外周血干细胞（PBSC）并进行冷冻保存。131I-MIBG通过固相铜离子催化的交换反应，将131I与127I-MIBG进行交换标记，在现场合成。131I-MIBG通过外周静脉在2小时内输注。剂量范围为557毫居里至1185毫居里（7.4毫居里/千克至18.75毫居里/千克）。中位剂量为833毫居里（12.55毫居里/千克）。骨髓发育不全在131I-MIBG治疗后3周开始。在首次131I-MIBG治疗后，19例患者需要输注血小板；19例接受粒细胞集落刺激因子（GCSF）；12例接受促红细胞生成素或红细胞。4例患者接受了PBSC输注。大剂量131I-MIBG在30例患者中产生了以下总体肿瘤反应：4例持续完全缓解（CR）；15例持续部分缓解（PR）；1例疾病稳定（SD）；5例疾病进展（PD）；5例最初为PR或SD但复发为PD。30例患者中有23例仍然存活；死亡原因分别为PD（5例）、骨髓发育异常（1例）和无关原因（1例）。5年总体预测生存率为75%（Kaplan-Meier估计）。对于转移性PHEO或PGL患者，在诊断性扫描中131I-MIBG摄取良好，大剂量131I-MIBG治疗对67%的患者有效，可产生持续的CR、PR或SD，且毒性可耐受。
