Surgical Management of Retroperitoneal Liposarcoma: Opportunities for Multimodality Treatment, Including Systemic Therapy

Abstract

Introduction: Soft tissue sarcomas are a diverse group of rare cancers, with approximately 15%-20% found in the retroperitoneum. Liposarcomas (LPS) make up approximately half of all retroperitoneal (RP) sarcomas, with most cases classified as either well-differentiated (WD) or dedifferentiated (DD). DD LPS is more aggressive, with a higher local recurrence rate and risk of distant metastasis compared to WD LPS. The purpose of this review is to outline surgical management of RP LPS and highlight the multimodal treatment strategies for both primary and recurrent disease, along with considerations for their effective implementation.

Methods: The current medical literature was reviewed for studies focused on retroperitoneal liposarcoma and its treatment with surgery, radiation, and chemotherapy. The data was interpreted and compiled in the context of expert clinical experience.

Results: Along with histopathologic analysis, tumor biology can inform patient prognosis. Surgery, the standard treatment for RP LPS, can be either curative or palliative. In primary disease, an attempt should be made to achieve wide surgical margins when feasible. Surgery for recurrent disease requires careful timing and an understanding of the potential benefit versus risk. Neoadjuvant radiation therapy can improve local control of RP LPS; however, data supporting the use of neoadjuvant chemotherapy are currently lacking.

Conclusion: Multimodality treatment of RP LPS is complex and requires consideration of tumor biology and extent of disease, along with individual patient characteristics. Multidisciplinary team collaboration is critical for improving outcomes in patients with RP LPS.

Keywords: dedifferentiated; liposarcoma; multimodality surgery; retroperitoneal sarcoma; surgery; well‐differentiated.

FIGURE 1

Case study: Primary LPS presentation and treatment. A 65‐year‐old male presented with a right‐sided retroperitoneal well‐differentiated LPS (A). He underwent compartmental resection of tumor with en bloc resection of right colon, right kidney, right adrenal gland, and ipsilateral retroperitoneal adipose tissue (B, C). LPS, liposarcoma. Figure courtesy of Kenneth Cardona; MD, FACS.

FIGURE 2

Case study: Recurrent LPS presentation and treatment. A 33‐year‐old female had surgery for primary WD LPS at age 26. She was found to have local recurrence 1.5 years later and subsequently underwent resection with en bloc radical nephrectomy, partial colectomy, and total ipsilateral retroperitoneal lipectomy. She developed local recurrence 2.9 years later and underwent re‐resection, sparing the duodenum and inferior vena cava. (A, B) One year later, she again developed local recurrence in the right hemiabdomen with multifocal tumors, including a biopsy‐proven dedifferentiated component (tumor 1). After multidisciplinary team discussion, she underwent neoadjuvant systemic therapy, initially with a CDK inhibitor. She quickly progressed and was switched to single‐agent doxorubicin. She then underwent her fourth resection, which included en bloc right hemicolectomy, as well as removal of several peripancreatic tumors. (C) Post‐resection field with all gross disease removed. (D) Surgical resection specimens shown corresponding to pre‐operative imaging (tumors 1–3). CDK, cyclin‐dependent kinase; LPS, liposarcoma; WD, well‐differentiated. Figure courtesy of Steven Sun, MD and William W. Tseng, MD.