Retroperitoneal sarcoma, an uncommon and challenging tumor.

Introduction: Soft tissue sarcoma may arise in any part of body and the most common site is extremities. Treatment of sarcoma is depended on multiple factors, including type, and site. When considering treatment of sarcoma, retroperitoneal sarcomas are treated in separated way. Difference is the especial location and adjacent organs in abdominal cavity. Methods and materials: We conducted a retrospective study in our center which is a referral center in south of Iran. Results: During a 6 year period, from 2006-2012 we had 101 cases of sarcoma among which, 12patients had retroperitoneal sarcoma. Mean age was 50.75(±16.80) years and most patients (9) were female. Adjuvant treatment was done for 11 patients that was chemotherapy in 3 and RT in 8 cases. The mean tumor size was 16 (±9.1) cm. 10 patients had clear margin, 1 patient had microscopic and 1 case had macroscopically involved margin. The mean RT dose was 3336.7 (±249.18) cGy. In those who received chemotherapy, chemotherapy regimens were, VAC. The mean DFS was 30.41 (the mean of follow up time was 51 (±32.35) months and the median of survival time was 68.81month 36.16). The mean of follow up time was49.16 (±37.83) month and the median of survival time was83.68month Discussion: treatment of sarcoma is complete tumor resection and in retroperitoneal area, a wide margin is not usually possible. Due to the rarity of these tumors and the complexity of treatment, evaluation and management should ideally be carried out in a center with multidisciplinary expertise in the treatment of sarcomas. In a review of four large series of patients with retroperitoneal sarcoma from institutions with extensive experience in management of this disease, complete resection rates were between 50 and 67 percent. In our center 10 patients had complete tumor resection. The five-year actuarial overall and disease-free survival and local control rates for all 37 patients were 50, 38, and 59 percent, respectively. In our center 5 year DFS and OS were 50%, 69%. Conclusion: results of our center are similar to other centers.

Keywords:

sarcoma , retroperitoneum , survival