Giant retroperitoneal sarcomas: a single institution experience

Retroperitoneal sarcomas (RS) are a rare group of malignant soft-tissue tumors; due to the flexibility of the retroperitoneum, they generally grow to a large size before becoming symptomatic, often involving surrounding structures. Therefore, the surgeon is frequently compelled to perform large excisions. The aim of this study is to assess clinical and pathological factors affecting prognosis in patients with RS who underwent surgical treatment, comparing giant forms (size > or = 25 cm) with smaller ones (size < 25 cm).

The hospital records of 73 consecutive patients who underwent surgical exploration for primary RS at our unit between 1984 and 2003 were reviewed. Statistical analysis of factors influencing overall and disease-free survival was performed including both the whole group of patients and only those who underwent complete surgical resection.

Giant RS showed a lower resectability rate than smaller forms (54.2% vs. 84.2%, P = 0.005). In the group with complete surgical excision (51 out of 73), patients with giant RS had a higher rate of adjacent organ resection compared with the smaller ones (84.2% vs. 53.1%, P = 0.023). Tumor size did not influence prognosis: after complete resection, 5-year overall survival was 60.9% and 56.3% for giant RS and smaller forms respectively, while 5-year disease-free survival was 54.3% and 48.3% for the two groups respectively. Advanced stage, incomplete gross surgical resection, higher tumor grade, non-liposarcoma histology and microscopic infiltration of margins were found to be significantly negative prognostic factors.

The present study confirms the importance of aggressive surgical management for RS, in order to offer these patients the best chance of long-term survival.