肽受体放射性核素治疗转移性神经内分泌肿瘤引起的异位库欣综合征。
Peptide receptor radionuclide therapy for ectopic Cushing's syndrome caused by metastatic neuroendocrine neoplasms.
作者信息
Boehm Emma, Hung Terry, Akhurst Tim, Alipour Ramin, Chiang Cherie, Hicks Rodney J, Hofman Michael S, Ravi Kumar Aravind S, Sachithanandan Nirupa, Saghebi Javad, Michael Michael, Kong Grace
机构信息
Department of Molecular Imaging and Therapeutic Nuclear Medicine, Peter MacCallum Cancer Centre, Melbourne, Australia.
Department of Internal Medicine, Endocrinology, Peter MacCallum Cancer Centre, Melbourne, Australia.
出版信息
Endocr Oncol. 2024 Nov 20;4(1):e240013. doi: 10.1530/EO-24-0013. eCollection 2024 Jan 1.
BACKGROUND
Metastatic gastroenteropancreatic neuroendocrine neoplasms (GEPNEN) can cause ectopic Cushing's syndrome (ECS). ECS is highly morbid and medical therapy is complex and can be ineffective. Patients unsuitable for bilateral adrenalectomy (BA) have dismal outcomes. Peptide receptor radionuclide therapy (PRRT) is a rational option for hormone and disease control in ECS caused by NEN with high somatostatin receptor (SSTR) expression.
AIM
To describe the characteristics and outcomes of patients with ECS treated with PRRT.
METHODS
Single-centre, retrospective analysis of imaging, biochemistry and outcomes of seven consecutive patients with ECS caused by metastatic GEPNEN treated with PRRT from 2006 to 2023.
RESULTS
Patients were aged 17-75 (female = 6). The primary site was the pancreas (5/7) and rectum (2/7). Six patients were on medical therapy for ECS at baseline (one had a previous BA). A median of 34.4 GBq of [Lu]Lu-DOTA-octreotate was given. [Y]Y-DOTA-octreotate (one patient) and [In]In-octreotide (one patient) were also used. Five patients had radiosensitising chemotherapy. Five patients had a flare of ECS within 1 week of PRRT cycle 1 (PRRT-C1). Following PRRT-C1, 5/7 patients had complete biochemical resolution of ECS at 1.5-6 months (four ongoing; one recurred after 12 months and had elective BA at 18 months). Best metabolic response on [F]F-FDG PET/CT: Four patients had a complete metabolic response (CMR), and one had a partial metabolic response. PFS was 3-208 months. Two patients progressed at the first follow-up. The longest ECS remission and CMR continues at >17 years.
CONCLUSION
PRRT can be effective for ECS caused by metastatic SSTR-positive GEPNEN and should be considered in its treatment algorithm.
背景
转移性胃肠胰神经内分泌肿瘤（GEPNEN）可导致异位库欣综合征（ECS）。ECS的发病率很高，药物治疗复杂且可能无效。不适合双侧肾上腺切除术（BA）的患者预后不佳。肽受体放射性核素治疗（PRRT）是控制由高生长抑素受体（SSTR）表达的神经内分泌肿瘤引起的ECS中的激素和疾病的合理选择。
目的
描述接受PRRT治疗的ECS患者的特征和结局。
方法
对2006年至2023年期间连续7例因转移性GEPNEN导致ECS并接受PRRT治疗的患者的影像学、生化指标和结局进行单中心回顾性分析。
结果
患者年龄为17 - 75岁（女性 = 6例）。原发部位为胰腺（5/7）和直肠（2/7）。6例患者在基线时接受ECS的药物治疗（1例曾接受BA）。给予的[¹⁷⁷Lu]Lu - DOTA - 奥曲肽的中位剂量为34.4 GBq。还使用了[⁹⁰Y]Y - DOTA - 奥曲肽（1例患者）和[¹¹¹In]In - 奥曲肽（1例患者）。5例患者接受了放射增敏化疗。5例患者在PRRT第1周期（PRRT - C1）的1周内出现ECS症状加重。PRRT - C1后，5/7的患者在1.5 - 6个月时ECS的生化指标完全缓解（4例持续缓解；1例在12个月后复发，18个月时接受了择期BA）。[¹⁸F]F - FDG PET/CT的最佳代谢反应：4例患者有完全代谢反应（CMR），1例有部分代谢反应。无进展生存期为3 - 208个月。2例患者在首次随访时病情进展。最长的ECS缓解期和CMR持续超过17年。
结论
PRRT对转移性SSTR阳性GEPNEN引起的ECS可能有效，应在其治疗方案中予以考虑。
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