How to treat liposarcomas located in retroperitoneum

• Published in: European journal of surgical oncology Vol. 49; no. 6; pp. 1068 - 1080
• Main Authors: Danieli, Maria, Swallow, Carol J., Gronchi, Alessandro
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01.06.2023
• Subjects: Chemotherapy; Humans; Lipopolysaccharides - therapeutic use; Liposarcoma; Liposarcoma - pathology; Liposarcoma - therapy; Neoadjuvant Therapy; Radiotherapy; Retroperitoneal Neoplasms - surgery; Retroperitoneal sarcoma; Sarcoma; Surgery; Survival; Chemotherapy; Sarcoma; Surgery; Liposarcoma; Retroperitoneal sarcoma; Radiotherapy; Survival
• ISSN: 0748-7983; 1532-2157
• DOI: 10.1016/j.ejso.2022.04.020

Almost half of retroperitoneal (RP) sarcomas are liposarcomas (LPS). The large majority of RP LPS are either well-differentiated LPS (WDLPS) or dedifferentiated LPS (DDLPS), these latter further classified according to grading in G2 and G3 DDLPS. Surgery is the only potentially curative treatment to achieve local control and possibly cure in primary localized disease. Over the last decade, a better delineation of the different histology-specific patterns of failure and the development of nomograms predictors of outcome has led to a better management of these rare tumors, with a special focus on non-surgical treatments. Available evidences – although far from exhaustive – show that radiation therapy might have a role, if any, as neoadjuvant treatment in locally aggressive histologies (i.e. WDLPS and G2 DDLPS), while it does not seem beneficial for histologies with a higher metastatic risk (i.e. G3 DDLPS and leiomyosarcoma). Neoadjuvant chemotherapy, instead, can be considered to reduce the risk of distant metastasis while waiting for the results of an ongoing RCT (STRASS-2) evaluating its effect in these tumors. However, given the rarity of these diseases and the subsequent lack of strong evidences to guide treatment, outcome improvement in these patients remains a challenge. Patients' referral to a sarcoma center where a dedicated specialized multidisciplinary team tailor optimal treatment on a case-by-case basis is crucial to ensure these patients the best outcome. Refining available nomograms – e.g including molecular variables – and identifying predictors of response/toxicity to chemotherapy and immunotherapy might be significantly helpful in tailoring treatments to the patient's characteristics. Also, new systemic agents are eagerly awaited for improving the management further.