Peet Connie, Elmaraghi Caroline, Abdel-Aziz Tarek, Liang Huang Hian, Gains Jennifer E, Nguyen Trung, Wan Simon, Bomanji Jamshed B, Gaze Mark N
Department of Radiotherapy, University College London Hospitals NHS Foundation Trust, London, UK.
Department of Oncology, University College London Hospitals NHS Foundation Trust, London, UK.
Eur J Nucl Med Mol Imaging. 2025 Apr 24. doi: 10.1007/s00259-025-07247-6.
Paraganglioma, phaeochromocytoma and gastroenteropancreatic neuroendocrine tumours are rare in childhood. Molecular radiotherapy is one potential treatment for locally inoperable or metastatic disease. This study reviews the use and efficacy of molecular radiotherapy with both [I] meta iodobenzylguanidine (mIBG) and [Lu] DOTATATE in this patient group.
This is an observational cohort study of all patients aged less than 18 years with adult type metastatic neuroendocrine cancers treated with molecular radiotherapy from 2003 to 2023 in one national referral centre.
Twelve patients, six male and six female, were treated. The median age at diagnosis was 12 years 3 months (range 7 years 11 months to 15 years 5 months), and at first molecular radiotherapy treatment was 13 years 7 months (range 8 years 8 months to 16 years 2 months). Nine had paraganglioma or phaeochromocytoma, three had other neuroendocrine tumours. Three received [Lu] DOTATATE only, four received [I] mIBG only, and five received both radiopharmaceuticals. Three patients had rapid disease progression and died within a year. Following initial treatment of the others, two had a complete response, four had a partial response, one had stable disease, and two had a mixed response. Nine patients remain alive, at a median of 5 years 0 months (range 2 years 4 months to 21 years 5 months) after start of treatment.
Molecular radiotherapy can be beneficial, and may provide good disease control for long periods in a proportion of these patients. Combining different radiopharmaceuticals may be of value.
副神经节瘤、嗜铬细胞瘤和胃肠胰神经内分泌肿瘤在儿童期较为罕见。分子放射治疗是局部无法手术切除或转移性疾病的一种潜在治疗方法。本研究回顾了[I]间碘苄胍（mIBG）和[Lu] DOTATATE分子放射治疗在该患者群体中的应用及疗效。
这是一项对2003年至2023年在一个国家转诊中心接受分子放射治疗的所有年龄小于18岁的成人型转移性神经内分泌癌患者的观察性队列研究。
共治疗了12例患者，6例男性和6例女性。诊断时的中位年龄为12岁3个月（范围7岁11个月至15岁5个月），首次分子放射治疗时的年龄为13岁7个月（范围8岁8个月至16岁2个月）。9例患有副神经节瘤或嗜铬细胞瘤，3例患有其他神经内分泌肿瘤。3例仅接受[Lu] DOTATATE治疗，4例仅接受[I] mIBG治疗，5例接受了两种放射性药物治疗。3例患者疾病进展迅速，在一年内死亡。对其他患者进行初始治疗后，2例完全缓解，4例部分缓解，1例病情稳定，2例有混合反应。9例患者仍然存活，治疗开始后的中位生存时间为5年0个月（范围2年4个月至21年5个月）。
分子放射治疗可能有益，并且在一部分此类患者中可能长期提供良好的疾病控制。联合使用不同的放射性药物可能有价值。
