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胃肠胰腺神经内分泌癌(gastroenteropancreatic neuroendocrine carcinoma，GEP-NEC)是一种少见的低分化恶性肿瘤，预后不良。根治性手术治疗是GEP-NEC的首选治疗方案，但术后复发率高，且相当一部分患者发现时已不具备手术机会。因此，全身化疗在GEP-NEC综合治疗中的地位尤为重要。本文旨在系统综述GEP-NEC全身化疗的研究进展。“,”Gastroenteropancreatic neuroendocrine carcinoma (GEP-NEC) is a rare and poorly differentiated tumor with poor prognosis. Although radical surgical treatment is the most preferred treatment for GEP-NEC, postoperative recurrence rate is still high. Many patients diagnosed at the late stage lost the chances to receive the surgery treatment. Therefore, systemic chemotherapy plays a particularly important role in the comprehensive treatment of GEP-NEC. The purpose of this paper is to systematically summarize and review the research progress of GEP-NEC systemic chemotherapy.