Armeni Eleni, Branton Alexander, Porto Juliana, Mandair Dalvinder, Hayes Aimee R, Manta Aspasia, Navalkissoor Shaunak, Gnanasegaran Gopinath, Quigley Ann-Marie, Grossman Ashley B, Caplin Martyn, Toumpanakis Christos
Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free Hospital NHS Trust, London, United Kingdom.
Endocrine Unit, 2nd Propaedeutic Department of Internal Medicine, Research Institute & Diabetes Center, Medical School, University of Athens, Attikon University Hospital, Athens, Greece.
Endocr Oncol. 2025 Aug 26;5(1):e240083. doi: 10.1530/EO-24-0083. eCollection 2025 Jan.
Glucagonomas are rare islet cell tumours, accounting for 2% of such tumours, with an annual incidence of 0.01-0.1 per million. This study aimed to describe diagnostic characteristics and treatment outcomes in patients with glucagonoma from a major referral centre.
A retrospective case series included patients diagnosed with glucagonoma at the ENETS Centre of Excellence, Royal Free Hospital, London, UK.
Electronic patient records were reviewed to document baseline disease characteristics and treatment outcomes. Disease-free survival (DFS), progression-free survival (PFS), and overall survival (OS) were calculated using the Kaplan-Meier method.
Twenty patients (75% male, age 56.6 ± 11.6 years, mean Ki-67 index 7.3 ± 7, mean ± SD) were included; 50% had liver metastases at diagnosis. The median OS was 34 months (95% CI: 30.3-37.7). Median OS was 34, 9, and 71 months for patients with liver, lung, and skeletal metastases, respectively. At diagnosis, migratory necrolytic erythema was linked to poorer OS (22 months, 95% CI: 14.3-29.7). Median DFS following surgery was 25 months (95% CI: 3.5-46.5). For inoperable disease, Lutetium-177 (Lu)-DOTATATE peptide receptor radionuclide therapy (PRRT) demonstrated efficacy in disease control. Other treatments included somatostatin analogues, chemotherapy, and molecular-targeted agents.
Tumour grade, metastases, and NME at diagnosis influence OS in glucagonoma. Surgery is associated with the best PFS as first-line therapy, while Lu-DOTATATE PRRT effectively controls disease progression. Further studies are needed to optimise treatment sequencing for advanced glucagonoma.
胰高血糖素瘤是罕见的胰岛细胞瘤，占此类肿瘤的2%，年发病率为百万分之0.01 - 0.1。本研究旨在描述来自一家主要转诊中心的胰高血糖素瘤患者的诊断特征和治疗结果。
一项回顾性病例系列研究纳入了在英国伦敦皇家自由医院ENETS卓越中心被诊断为胰高血糖素瘤的患者。
回顾电子病历以记录基线疾病特征和治疗结果。采用Kaplan-Meier方法计算无病生存期（DFS）、无进展生存期（PFS）和总生存期（OS）。
纳入20例患者（75%为男性，年龄56.6±11.6岁，平均Ki-67指数7.3±7，均值±标准差）；50%在诊断时已有肝转移。中位总生存期为34个月（95%置信区间：30.3 - 37.7）。肝、肺和骨转移患者的中位总生存期分别为34、9和71个月。诊断时，游走性坏死性红斑与较差的总生存期相关（22个月，95%置信区间：14.3 - 29.7）。手术后的中位无病生存期为25个月（95%置信区间：3.5 - 46.5）。对于不可手术切除的疾病，镥-177（Lu）-奥曲肽肽受体放射性核素治疗（PRRT）在疾病控制方面显示出疗效。其他治疗包括生长抑素类似物、化疗和分子靶向药物。
肿瘤分级、转移情况以及诊断时的游走性坏死性红斑会影响胰高血糖素瘤的总生存期。手术作为一线治疗与最佳的无进展生存期相关，而Lu-奥曲肽PRRT可有效控制疾病进展。需要进一步研究以优化晚期胰高血糖素瘤的治疗顺序。
