Mayer Karin, Kiry Selina, Yordanova Anna, Ahmadzadehfar Hojjat, Gaertner Florian C, Bundschuh Ralph A, Essler Markus, Gonzalez-Carmona Maria A, Strassburg Christian P, Matthaei Hanno, Lingohr Philipp, Bisht Savita, Brossart Peter, Feldmann Georg
Department of Internal Medicine 3, Center of Integrated Oncology Aachen-Bonn-Cologne-Duesseldorf, University Hospital of Bonn, Bonn, Germany.
Department of Nuclear Medicine, Center of Integrated Oncology Aachen-Bonn-Cologne-Duesseldorf, University Hospital of Bonn, Bonn, Germany.
Int J Endocrinol. 2020 Jan 31;2020:1491475. doi: 10.1155/2020/1491475. eCollection 2020.
Neuroendocrine neoplasias (NENs) represent a rare and biologically heterogeneous group of malignancies. Treatment of NEN patients remains challenging due to lack of prospective evidence on the choice of ideal therapeutic sequence and therapeutic efficacy in specific individual scenarios.
Clinical data on 110 consecutive patients suffering from NEN treated at a single German university center were analyzed, therapeutic regimens applied were assessed, and the outcome was evaluated.
Histological grading, Ki67 proliferation index, functional activity, and presence of metastases were identified as prognostic markers. 10-year overall survival rates were 92%, 44%, and 0% for G1, G2, and G3 tumors, and 60%, 39%, 69%, 53%, and 0% for Ki67 <2%, 3-5%, 6-20%, 21-49%, and >50%, respectively. Peptide receptor radionuclide therapy (PRRT) and cytostatic chemotherapy were the second most common options, with PRRT being used more frequently in NET G1 and G2 and chemotherapy in NEC G3. Combination chemotherapy with etoposide plus cisplatin or carboplatin showed disease control rates (DCRs) of overall 74%, with a short median progression-free survival (PFS) of 7 or 5 months, respectively. DCR and PFS for PRRT were 89% and 22 months when administered as monotherapy, versus 100% and 27 months upon combination with somatostatin analog (SSA) therapy. Of note, PRRT also achieved disease control as best response in 5/5 (100%) selected cases of NEC G3.
Further prospective studies are warranted to help stratify available options for therapeutic intervention in NEN patients.
神经内分泌肿瘤（NENs）是一类罕见且生物学特性各异的恶性肿瘤。由于缺乏关于理想治疗顺序选择及特定个体情况下治疗效果的前瞻性证据，NEN患者的治疗仍然具有挑战性。
分析了在德国一所大学中心连续治疗的110例NEN患者的临床数据，评估了所应用的治疗方案，并对结果进行了评估。
组织学分级、Ki67增殖指数、功能活性和转移情况被确定为预后标志物。G1、G2和G3肿瘤的10年总生存率分别为92%、44%和0%，Ki67<2%、3 - 5%、6 - 20%、21 - 49%和>50%的患者10年总生存率分别为60%、39%、69%、53%和0%。肽受体放射性核素治疗（PRRT）和细胞毒性化疗是第二常见的选择，PRRT在NET G1和G2中使用更频繁，化疗在NEC G3中使用更频繁。依托泊苷联合顺铂或卡铂的联合化疗总体疾病控制率（DCR）为74%，中位无进展生存期（PFS）较短，分别为7个月或5个月。PRRT单药治疗时的DCR和PFS分别为89%和22个月，与生长抑素类似物（SSA）联合治疗时分别为100%和27个月。值得注意的是，PRRT在5/5（100%）例选定的NEC G3病例中也实现了疾病控制的最佳反应。
有必要进行进一步的前瞻性研究，以帮助对NEN患者的治疗干预可用选项进行分层。
