| Abstract: |
Purpose of Review: Primary retroperitoneal sarcoma can be difficult to treat due to variable biologic behavior of histologic subtypes, size of tumors and involvement of adjacent structures. We provide an overview of current treatment algorithms employed to manage these tumors. Recent Findings: Diagnosis of retroperitoneal sarcomas requires cross-sectional imaging to assess for pathognomonic characteristics and, often, biopsy (with immunohistochemical and molecular assessment) to define histologic subtyping (e.g., well-, or dedifferentiated liposarcomas, leiomyosarcomas). To optimize outcomes, treatment at specialty centers includes evaluation by multidisciplinary tumor boards though aggressive surgical resection remains the backbone of therapy. Careful follow-up after resection often detects local or distal recurrence, rates of which are defined by tumor subtype. A randomized clinical trial of neoadjuvant radiation in primary disease (STRASS) showed no benefit in terms of abdominal recurrence, but radiation and systemic therapies can be useful in management of marginally resectable and metastatic disease. Ongoing studies suggest a role for novel targeted and immunotherapies for a subset of cases (e.g., CDK4 and PD-L1 inhibitors in dedifferentiated liposarcomas). Summary: Although surgical resection is the backbone of retroperitoneal sarcoma therapy, patients benefit from a multidisciplinary, subtype-tailored approach with non-operative treatments increasingly employed to optimize patient outcomes. © The Author(s) 2024. |
