Retroperitoneal sarcomas are a group of diseases that behave differently from one another. Well-differentiated liposarcomas have an indolent biology but show a tendency to recur locally even years after primary resection. Dedifferentiated liposarcomas are characterized by a very high local recurrence risk, while the metastatic risk mainly depends on the histological characteristics of the dedifferentiated component. In leiomyosarcomas, hematogenous spread informs prognosis while local recurrences are far less common. Surgery is the cornerstone of treatment of all retroperitoneal sarcoma subtypes and its quality is the only treatment-related factor able to improve the oncological outcome. A frontline extended surgical approach is all the more critical in subtypes in which local control directly impacts prognosis.
Keywords: leiomyosarcoma; liposarcoma; outcome; prognosis; retroperitoneal sarcoma; sarcoma; solitary fibrous tumor; surgery; surgical margins.