Introduction: Pheochromocytomas and paragangliomas are rare neuroendocrine tumours that originate from chromaffin cells within the adrenal medulla or extra-adrenal sympathetic ganglia. Management of disseminated or metastatic pheochromocytomas and paragangliomas continues to pose challenges and relies on limited evidence. Method: In this study, we report retrospective data on median overall survival (OS) and median progression-free survival (PFS) for all Danish patients treated with peptide receptor radionuclide therapy (PRRT) with177Lu-Dotatate or90Y-Dotatate over the past 15 years. One standard treatment of PRRT consisted of 4 consecutive cycles with 8–14-week intervals. Results: We included 28 patients; 10 were diagnosed with pheochromocytoma and 18 with paraganglioma. Median age at first PRRT was 47 (IQR 15–76) years. The median follow-up time was 31 (IQR 17–37) months. Eight patients died during follow-up. Median OS was 72 months, and 5-year survival was 65% with no difference between pheochromocytoma and paraganglioma. Patients with germline mutations had better survival than patients without mutations (p= 0.041). Median PFS after the first cycle of PRRT was 30 months. For patients who previously received systemic treatment, the median PFS was 19 months, compared with 32 months for patients with no previous systemic treatment (p= 0.083). Conclusions: The median OS of around 6 years and median PFS of around 2.5 years found in this study are comparable to those reported in previous studies employing PRRT. Based on historical data, the efficacy of PRRT may be superior to131I-MIBG therapy, and targeted therapy with sunitinib and PRRT might therefore be considered as first-line treatment in this patient group.
引言：嗜铬细胞瘤和副神经节瘤是起源于肾上腺髓质或肾上腺外交感神经节嗜铬细胞的罕见神经内分泌肿瘤。播散性或转移性嗜铬细胞瘤及副神经节瘤的治疗仍面临挑战，且现有循证依据有限。方法：本研究回顾性分析了丹麦过去15年间接受肽受体放射性核素治疗（采用177Lu-Dotatate或90Y-Dotatate）患者的总体生存期与无进展生存期数据。标准PRRT方案为连续4个治疗周期，间隔8-14周。结果：共纳入28例患者，其中嗜铬细胞瘤10例，副神经节瘤18例。首次PRRT治疗中位年龄为47岁（四分位距15-76岁）。中位随访时间31个月（四分位距17-37个月）。随访期间8例患者死亡。中位总体生存期为72个月，5年生存率65%，两种肿瘤类型间无统计学差异。携带种系突变患者较无突变者生存期更优（p=0.041）。首次PRRT治疗后中位无进展生存期为30个月。既往接受过全身治疗患者的中位无进展生存期为19个月，未接受过全身治疗者为32个月（p=0.083）。结论：本研究发现的约6年中位总体生存期与2.5年中位无进展生存期，与既往PRRT研究结果具有可比性。基于历史数据，PRRT疗效可能优于131I-MIBG治疗，因此舒尼替尼靶向治疗联合PRRT可考虑作为该患者群体的一线治疗方案。
肿瘤(癌症)患者之家