目的探讨小肠原发性恶性肿瘤的临床特点。方法通过14例病例并结合文献报道,分析小肠原发性恶性肿瘤的临床资料。结果原发性小肠腺癌以肠梗阻的症状为主要表现,原发性小肠平滑肌肉瘤和恶性淋巴瘤则以腹痛、腹块为主要表现;从出现首发症状到发现病变的时间为1.5～24个月;检查手段以CT、血管造影及小肠镜为优,B超和钡剂小肠造影诊断价值有限;治疗以手术切除为主,化疗或放疗可作为手术后辅助治疗,本组根治切除率为78.6%。结论小肠原发性恶性肿瘤早期诊断较困难,检查以CT、血管造影及小肠镜检为主,根治性切除为首选治疗手段。 Objective To investigate the clinical features of primary small intestine malignancies. Methods The clinical data of primary small intestine were analyzed through 14 cases and reported in the literature. Results Primary intestinal adenocarcinoma showed the symptoms of intestinal obstruction as the main manifestation. Primary small bowel leiomyosarcoma and malignant lymphoma mainly belonged to abdominal pain and abdominal mass. The time from onset of first symptom to finding of lesions ranged from 1.5 to 24 Month; check the means of CT, angiography and enteroscopy as the best, B ultrasound and barium small intestinal angiography is of limited diagnostic value; the main surgical resection, chemotherapy or radiotherapy can be used as postoperative adjuvant therapy, the group of radical resection rate was 78.6 %. Conclusion Early diagnosis of primary small intestinal malignant tumor is more difficult to check the CT, angiography and small intestine microscopy mainly radical resection as the preferred treatment.