ASO Author Reflections: Recurrent Retroperitoneal Well-Differentiated Liposarcoma

Excerpt

Sarcomas are rare tumors representing 0.7% of all cancer cases in adults, and approximately 15–20% of those occur in the retroperitoneum.1 Sarcoma is an extremely heterogeneous disease group that encompasses > 60 histologies; however, because it is a rare disease group, retroperitoneal sarcomas (RPSs) had been historically treated and studied as one group entity. Analysis of 1118 patients with RPSs treated at MD Anderson Cancer Center showed significant variability in 5-year overall survival (OS) (well-differentiated liposarcoma [WDLPS], 95%; other liposarcomas [mostly dedifferentiated liposarcoma], 25%, and other histology [mostly leiomyosarcoma], 43%).2 Clear differences in recurrence pattern by tumor histology also were described in recent large retrospective studies. Liposarcomas have high local recurrence rates (35–60%) and a high metastasis rate only if dedifferentiated (30%), whereas leiomyosarcoma has a high distant metastasis rate (60%) and a low local recurrence rate (< 20%).3,4 These reports described the importance of histology-driven decision making for the treatment strategy of RPSs, both in the management of recurrent disease as well as that of primary tumors. …