Satapathy Swayamjeet, Mittal Bhagwant Rai, Bhansali Anil
Department of Nuclear Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Endocrinology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Clin Endocrinol (Oxf). 2019 Dec;91(6):718-727. doi: 10.1111/cen.14106. Epub 2019 Oct 13.
Inoperable and metastatic pheochromocytomas and paragangliomas (PPGLs) present a therapeutic challenge with current treatment options being limited to radiolabelled meta-iodo-benzyl-guanidine (MIBG) and systemic chemotherapy. Peptide receptor radionuclide therapy (PRRT) seems to be a promising option for these patients with few studies reporting favourable response. This systematic review was conducted to evaluate the efficacy and safety of PRRT in patients with advanced PPGLs.
This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Searches in PubMed, Scopus and Embase were made using relevant keywords and articles up to May 2019 were included. Data on efficacy and toxicity were extracted from the individual articles, and pooled estimates were generated using meta-analysis.
Twelve articles consisting of 201 patients with advanced PPGLs were included. Overall, treatment with PRRT achieved an objective response rate of 25% (95% CI: 19%-32%) and a disease control rate of 84% (95% CI: 77%-89%). Clinical and biochemical responses were seen in 61% and 64% of the patients, respectively. Among the PRRTs, similar tumour response rates were noted for Y-yttrium- and Lu-lutetium-based agents. Treatment-related adverse effects were minimal with grade 3/4 neutropenia, thrombocytopenia, lymphopenia and nephrotoxicity observed in 3%, 9%, 11% and 4% of the patients, respectively. Treatment discontinuation was noted in five out of 102 patients.
Peptide receptor radionuclide therapy is a safe and efficacious treatment option for advanced PPGLs and may be considered a viable alternative to chemotherapy and I- MIBG.
无法手术切除的转移性嗜铬细胞瘤和副神经节瘤（PPGLs）带来了治疗挑战，目前的治疗选择仅限于放射性标记的间碘苄胍（MIBG）和全身化疗。肽受体放射性核素治疗（PRRT）似乎是这些患者的一个有前景的选择，不过仅有少数研究报告了良好的反应。本系统评价旨在评估PRRT在晚期PPGLs患者中的疗效和安全性。
本评价遵循系统评价和Meta分析的首选报告项目（PRISMA）指南。在PubMed、Scopus和Embase中进行检索，使用相关关键词并纳入截至2019年5月的文章。从各篇文章中提取疗效和毒性数据，并使用Meta分析生成汇总估计值。
纳入了12篇文章，共201例晚期PPGLs患者。总体而言，PRRT治疗的客观缓解率为25%（95%CI：19%-32%），疾病控制率为84%（95%CI：77%-89%）。分别有61%和64%的患者出现临床和生化反应。在基于钇-90和镥-177的PRRT中，观察到相似的肿瘤反应率。与治疗相关的不良反应轻微，分别有3%、9%、11%和4%的患者出现3/4级中性粒细胞减少、血小板减少、淋巴细胞减少和肾毒性。102例患者中有5例停止治疗。
肽受体放射性核素治疗是晚期PPGLs的一种安全有效的治疗选择，可被视为化疗和131I-MIBG的可行替代方案。
