Objective To investigate the clinical and pathological characteristics,surgical treatment strategy and prognosis of primary malignant neoplasms of the appendix.Methods The clinical data of 74 patients with primary malignant neoplasms of the appendix in our hospital from January 1982 to December 2012 were retrospectively studied.Results Among the 74 cases of primary malignant neoplasms of the appendix,carcinoids were the most common accounting for approximately 70％,adenocarcinoma accounting for 22％ and lymphoma accounting for 8％.The prognosis of primary malignant neoplasms of the appendix is rather poor,nainly because of patients' later presentetion.The overall 1,3,5-year survival rate is respectively 95％,74％,60％,the prognosis of carcinoid is good,while that of adenocarcinoma is poor.Conclusions The incidence of primary malignant neoplasms of the appendix is relatively low.It is difficult to diagnose preoperatively,and the diagnosis relies mainly on rapid intraoperative frozen section and postoperative pathology.