Abstract
Simple SummaryThis review discusses current and prospective treatment strategies for retroperitoneal liposarcoma, a rare type of sarcoma with a high propensity for locoregional recurrence and low survival rate. Chemo- and radiotherapy regimens, as well as molecular targets, are highlighted as important tools to better explore the mechanisms underlying this disease and to pursue new possible targetable pathways.Liposarcoma (LPS) is the most prevalent soft tissue sarcoma histological subtype. When it occurs in the abdomen the overall survival rate is as low as 10% at 10 years and is fraught with high rates of recurrence, particularly for the more aggressive dedifferentiated subtype. Surgery remains the mainstay of treatment. Systemic therapies for the treatment of metastatic or unresectable disease have low response rates. Deep understanding of well-differentiated and de-differentiated LPS (WDLPS and DDLPS, respectively) oncologic drivers is necessary for the development of new efficacious targeted therapies for the management of this disease. This review discusses the current treatments under evaluation for retroperitoneal DDLPS and the potential targetable pathways in DDLPS.
Highlights
Soft-tissue sarcomas (STS) are a heterogenous group of tumors that represent about1% of all adult malignancies [1]
One recent phase III randomized controlled trial, the STRASS-1 trial (Surgery With or Without Radiation Therapy in Untreated Nonmetastatic Retroperitoneal Sarcoma), evaluated oncologic outcomes in patients with RPS who underwent neoadjuvant radiation followed by surgery versus surgery alone [21]
No statistically significant difference in recurrence-free survival (RFS) was seen among the two groups (3-year RFS was 60.4% in the radiation group vs. 58.7% in the surgery-only group), there was a trend suggesting that certain histologic subtypes, such as well-differentiated liposarcoma (WDLPS) and low-grade de-differentiated liposarcoma (DDLPS), may benefit from neoadjuvant radiation [1,21]
Summary
Soft-tissue sarcomas (STS) are a heterogenous group of tumors that represent about. 1% of all adult malignancies [1]. Retroperitoneal liposarcomas (RPLPS) represent one of the most common histologic subtypes, with their own distinct biology and high risk for local versus distant recurrence [2,3]. Myxoid liposarcoma is the second most common subtype, and it represents about 5% of all soft tissue sarcomas in adults [5]. The EORTC 62,012 trial demonstrated that liposarcomas responded better to chemotherapy than other sarcoma subtypes [16] Second line agents such as trabectedin have been studied, but these have been found to be primarily beneficial among the myxoid liposarcoma histologic subtype (Table 1) [17]. RPS: Retroperitoneal sarcoma; RT: Radiotherapy; IG-IMPT: Image Guided Intensiy Modulated Proton Radiation Therapy; IG-IMRT: Image Guided Intensity Modulated Photon Radiation Therapy; DFS: Disease-free survival; PFS: Progression-free survival; RFS: Recurrence-free survival; STS: Soft Tissue Sarcoma; LMS: Leiomyosarcoma
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