Abstract: Objective:To investigate the clinical features, therapeutic method, and prognostic factor of fibrosarcomatous dermato-fibrosarcoma protuberans (FS- DFSP). Methods:Data of18FS- DFSP cases treated in the Department of General Surgery, Tianjin Huanghe Hospital between June2004and June 2014were retrospectively analyzed. Research indexes included age, sex, pathogenic site, number of previous unplanned surgeries, tumor size, depth, incisal margin of the last surgical procedure, chemotherapy, relapse and metastasis, and survival time. Univariate and multivariate analyses of the research indicators were conducted in these cases.Results: All18patients underwent surgery, and the incisal margins were R 0 in 17cases and R 1 in 1 case, with local recurrence in 2 of the 18cas-es. The patient with R1 incisal margin underwent postoperative radiotherapy. Among all the patients, 12with tumor base depth and/or largest tumor diameter of >5 cm accepted the chemotherapy of mesna, adriamycin, ifosfamide, and dacarbazine, also known as MAID regimen, after surgery. No progression of disease occurred during chemotherapy. After chemotherapy, pulmonary metastasis occurred in 3 of the 12cases, and pulmonary metastasis with local recurrence existed in 1 case. Two of the 3 patients with pulmonary metastasis were treated with imatinib mesylate, and the therapeutic effect stabilized the disease. Two-year survival rate was 93%, and 5-year sur -vival rate was 79% in total patients. The results of mono-factorial analysis indicated that clinical factors, such as age, sex, pathogenic site, tumor size, depth, recurrence, incisal margin of surgical operation, and chemotherapy, were unrelated to the overall survival (OS) time. The number of previous unplanned surgeries and metastasis are related to OS. The results of multiple factor analysis showed that none of the clinical factors were independent prognostic factors for OS. Conclusion:A thorough removal of tumor can reduce the recur-rence rate, which is the key point in FS-DFSP treatment. Recurrence and metastasis of tumor are significant factors affecting prognosis.