Role of chemotherapy in dedifferentiated liposarcoma of the retroperitoneum: defining the benefit and challenges of the standard

Abstract

Benefit from chemotherapy for well-differentiated/de-differentiated (WD/DD) liposarcomas has been reported to be minimal, however traditional response criteria may not adequately capture positive treatment effect. In this study, we evaluate benefit from first-line chemotherapy and characterize imaging response characteristics in patients with retroperitoneal (RP) WD/DD liposarcoma treated at The University of Texas MD Anderson Cancer Center. Response was assessed using RECIST (Response Evaluation Criteria in Solid Tumors) and an exploratory analysis of vascular response was characterized. Among 82 patients evaluable for response to first-line therapy, 31 patients received neoadjuvant chemotherapy for localized/locally advanced disease; 51 received chemotherapy for unresectable recurrent/metastatic disease. Median overall survival from the start of chemotherapy was 29 months (95% CI 24–40 months). Response rates by RECIST: partial response (PR) 21% (17/82), stable disease (SD) 40%, and progression (PD) 39%. All RECIST responses were in patients receiving combination chemotherapy. A qualitative vascular response was seen in 24 patients (31%). Combination chemotherapy yields a response rate of 24% and a clinical benefit rate (CR/PR/SD > 6 months) of 44%, higher than previously reported in DD liposarcoma. A higher percentage of patients experience a vascular response with chemotherapy that is not adequately captured by RECIST in these large heterogeneous tumors.

Resection outcomes for primary and local recurrent retroperitoneal liposarcoma patients

“…These studies shown that preoperative neoadjuvant radiotherapy can prolong the survival of RPLPS, especially in patients with high-risk pathological types [22], and reduce the possibility of local recurrence [23]. MD Anderson Cancer Center reported that combined chemotherapy in patients with RP DDLPS produced a response rate of 24% [24]. Since new adjuvant treatment methods have not been widely practiced in the clinic, they are given to refractory or complicated liposarcoma cases.…”

Surgical Management of a Breast Liposarcoma Arising in An Elderly Male with An Overlying Permanent Pacemaker: A Case Report with Associated Radiologic and Intraoperative Imaging

“…Liposarcoma is the most common soft-tissue sarcoma encountered, with dedifferentiated liposarcoma usually arising in the retroperitoneum [4,5]. 10 % of well-differentiated liposarcomas can have a component of dedifferentiation.…”

“…MDM2 and CDK4 is positive in dedifferentiated liposarcoma and negative in myxoid and pleomorphic liposarcomas. Supernumerary rings and/or giant rod chromosomes that contain amplified sequences of the region 12q14-15 are characteristic of dedifferentiated liposarcoma [5].…”

Overview of liposarcomas and their genomic landscape

“…For patients with primary localized LPS, surgery with complete gross tumor resection (R0/R1 margins) remains the definitive management and only potential curative treatment. However, local recurrence rates are high (> 80%) [53,54] . Radiation therapy (RT) and/or chemotherapy can be used as adjuncts to reduce local and distant recurrence risk for those with dedifferentiated, myxoid/round cell, or pleomorphic LPS.…”

“…Cytotoxic therapies WDLPS and DDLPS are relatively chemoresistent [1,2,55] , with response rates in the literature reported as low as ≤ 12% [56] and as high as 21% [53] [ Table 1]. Thus, in the primary or recurrent resectable setting, systemic therapy has not been frequently used and there is no consensus regarding their use in the neoadjuvant or adjuvant setting.…”