Modern multimodality management of patients with caval leiomyosarcoma: New treatment paradigms and potential
Malcolm H Squires1, Stephen Politano2, Raphael E Pollock2
1Division of Surgical Oncology, Department of Surgery, Levine Cancer Institute, Atrium Health, Charlotte, North Carolina, USA.
Background And Objectives:
Caval leiomyosarcomas (cLMS) are rare soft tissue sarcomas historically associated with high recurrence rates and poor prognosis. While radical resection remains the mainstay of therapy for cLMS, new systemic therapies have presented opportunities for multimodality treatment. We examined the clinical outcomes of patients with cLMS treated with modern, multimodality approaches, and compared their outcomes to those of patients with noncaval retroperitoneal LMS (ncLMS).
Methods:
A retrospective, single-institution review identified all patients diagnosed with primary retroperitoneal LMS from 2012 to 2018. Radiographic and pathologic review distinguished patients with cLMS and ncLMS. Standard clinicopathologic variables and response to chemotherapy (when applicable) were analyzed. Primary endpoints were overall (OS) and progression-free survival (PFS).
Results:
Eleven patients with cLMS were identified. Median tumor size was 7.5 cm (IQR, 5.0-14.3 cm); all patients had Stage II/III disease. Seven patients received neoadjuvant chemotherapy. Nine cLMS patients underwent R0/R1 resection; two did not complete resection. Six patients received adjuvant systemic therapy. Twenty patients with ncLMS were treated during the same period. No statistical intergroup differences were noted in tumor size, pathologic grade, stage, or resection margin status. Patients with ncLMS were less likely to receive neoadjuvant (10% vs. 64%) and adjuvant chemotherapy (30% vs. 55%). Two-year OS (81% vs. 78%; p = NS) and PFS (55% vs. 46%; p = NS) were comparable between cLMS and ncLMS patients.
Conclusions:
Multimodality treatment with systemic therapy and aggressive surgical resection may achieve equivalent survival outcomes for patients with cLMS versus similar ncLMS. We recommend that all patients with cLMS be evaluated for multidisciplinary treatment. Genomic and proteomic expression profiling may identify novel or targetable mutations.
Insights
Multimodality treatment combining chemotherapy and surgery offers comparable survival outcomes for patients with caval leiomyosarcomas (cLMS) and noncaval retroperitoneal LMS (ncLMS). Multidisciplinary evaluation is recommended for all cLMS patients.
Area of Science:
- Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Caval leiomyosarcomas (cLMS) are rare, aggressive soft tissue sarcomas with historically poor prognoses and high recurrence rates.
- Radical surgical resection is the primary treatment for cLMS, but advancements in systemic therapies enable multimodality treatment approaches.
Purpose of the Study:
- To evaluate the clinical outcomes of patients with cLMS treated with modern multimodality approaches.
- To compare the outcomes of cLMS patients with those of patients diagnosed with noncaval retroperitoneal LMS (ncLMS).
Main Methods:
- A retrospective, single-institution review of primary retroperitoneal LMS cases diagnosed between 2012 and 2018.
- Patients were categorized into cLMS and ncLMS groups based on radiographic and pathologic review.
- Analysis of clinicopathologic variables, treatment response, overall survival (OS), and progression-free survival (PFS).
Main Results:
- Eleven cLMS patients and twenty ncLMS patients were identified, with no significant differences in tumor size, grade, stage, or resection margins.
- A higher proportion of cLMS patients received neoadjuvant (64% vs. 10%) and adjuvant (55% vs. 30%) chemotherapy compared to ncLMS patients.
- Two-year OS (81% vs. 78%) and PFS (55% vs. 46%) were comparable between the cLMS and ncLMS groups.
Conclusions:
- Multimodality treatment, including systemic therapy and aggressive resection, may yield survival outcomes for cLMS comparable to ncLMS.
- Multidisciplinary evaluation is recommended for all patients with cLMS to optimize treatment strategies.
- Genomic and proteomic profiling may reveal novel therapeutic targets for cLMS.
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