Diagnosis and management of dedifferentiated liposarcoma: A multidisciplinary position statement

Abstract

Dedifferentiated liposarcoma (DDLPS) is a malignant mesenchymal neoplasm in desperate need of novel therapeutic approaches. Often occurring in conjunction with well-differentiated liposarcoma (WDLPS), DDLPS can behave more aggressively and exhibits a significant risk for developing recurrence or metastatic disease when compared to its well-differentiated counterpart. A multidisciplinary approach is critically important, particularly for patients with localized disease, as disease presentations are often complex, and the management of patients has become increasingly nuanced as treatment approaches have become more refined. Expert pathology review and appropriate application of diagnostic molecular techniques are key components of DDLPS diagnosis and also reflect an improved understanding of the underlying pathogenesis of the disease. Systemic therapies remain limited for DDLPS, but novel therapies targeting important underlying molecular drivers have resulted in ongoing clinical trials aiming to improve outcomes for patients with advanced disease. In recognition of the increased activity and interest within the DDLPS field, a multidisciplinary group of nationally recognized experts in medical oncology, surgical oncology, radiation oncology, and pathology was convened to summarize key insights. This position paper highlights important points from the meeting and provides evidence-based recommendations for practicing clinicians.