胰腺神经内分泌肿瘤的流行病学、诊断学及药物治疗研究进展

Advances in Epidemiology, Diagnostics and Drug Treatment of Pancreatic Neuroendocrine Neoplasm

摘要: 胰腺神经内分泌肿瘤是来源于弥散的胰腺神经内分泌细胞，被认为是一种罕见的胰腺肿瘤。随着医学影像学、基因组学、免疫组织病理学等相关学科的发展，胰腺神经内分泌肿瘤的正确诊断率及检出率越来越高。胰腺神经内分泌肿瘤被正确地诊断及病理学分级对患者后续治疗选择是至关重要的。此外，胰腺神经内分泌肿瘤的治疗与肿瘤分期、肿瘤分级分化、激素产生、肿瘤负荷和肿瘤进展高度相关。随着分子靶向药物和生长抑素类似物的问世，胰腺神经内分泌肿瘤的药物治疗取得了很大进展。目前，全身治疗药物方案包括生长抑素类似物(SSAs)、分子靶向药物、细胞毒性化疗药物、免疫检查点抑制剂和肽受体放射性核素治疗(PRRT)。已被临床使用和正在进行临床试验的药物都显著提高了胰腺神经内分泌肿瘤患者的生存率。本文就胰腺神经内分泌肿瘤的流行病学、诊断学及药物治疗的最新进展做一综述。

Abstract: Pancreatic neuroendocrine neoplasm is derived from diffused pancreatic neuroendocrine cells and is considered to be a rare pancreatic tumor. With the development of medical imaging, genomics, immunohistopathology and other related disciplines, the correct diagnosis rate and detection rate of pancreatic neuroendocrine neoplasm are getting higher and higher. The correct diagnosis and pathological grading of pancreatic neuroendocrine neoplasm are very important for the choice of subsequent treatment. In addition, the treatment of pancreatic neuroendocrine neoplasm is highly correlated with tumor stage, tumor grade and differentiation, hormone production, tumor burden and tumor progression. With the advent of molecular targeted drugs and somatostatin analogues, great progress has been made in the drug therapy of pancreatic neuroendocrine neoplasm. At pre-sent, systemic therapeutic drug regimens include somatostatin analogues (SSAs), molecular tar-geted drugs, cytotoxic chemotherapeutic drugs, immune checkpoint inhibitors and peptide recep-tor radionuclide therapy (PRRT). Drugs in clinical use and ongoing clinical trials have significantly improved survival in patients with pancreatic neuroendocrine neoplasm. This article reviews the latest advances in epidemiology, diagnostics and drug treatment of pancreatic neuroendocrine ne-oplasm.