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摘要: 近年许多分子标志物与影像学诊断技术应用于胰腺神经内分泌肿瘤(PNET)的诊断,大幅提高了PNET的诊断准确性。在PNET的治疗方面,手术切除仍是唯一具有治愈可能的治疗方式,根据肿瘤的类型、部位、大小情况可以选择不同的手术方式。而传统的化疗由于不良反应大等原因,临床应用有限。但是随着对PNET发病机制的了解,生长抑素类似物、血管生成抑制剂、mTOR抑制剂等靶向药物已经应用于临床,为PNETs患者带来了希望。Abstract: Pancreatic neuroendocrine tumors (PNETs) have an annual incidence of approximately 1 per 100, 000 individuals and most often occur in adults aged 40 years to 60 years. PNETs, also called islet cell tumors, originate from the Langerhans cells in the pancreatic islet. Based on the presence or absence of specific hormone-related symptoms, PNETs are classified as either functional or non-functional. Depending on their clinicopathological characteristics, these tumors can also be classified as G1, G2, or G3 in the World Health Organization classification and grading system. The American Joint Committee on Cancer and European Neuroendocrine Tumor Society has also introduced the TNM staging classification for PNETs. New technologies, including molecular markers and medical imaging, have been used to diagnose PNET and have significantly improved the diagnostic accuracy. As for the treatment of PNET, surgery may be the only option. Different modes of surgery can be used depending on the tumor type, location, and size. Clinical application of traditional chemotherapeutic agents is limited because of their toxicity. However, with the increased understanding of PNET pathogenesis, therapeutic agents such as somatostatin analogs, angiogenesis inhibitors, and mTOR inhibitors have already been used in clinical practice and have brought hope to patients.
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Key words:
- neuroendocrine tumors /
- diagnosis /
- surgery /
- targeted therapy
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表 1 AJCC与ENETS的TNM分期
Table 1. TNM staging classification for AJCC and ENETS
表 2 功能性PNET诊断标准
Table 2. Diagnostic criteria for functional pancreatic neuroendocrine tumor
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