The use of pegylated liposomal doxorubicin in metastatic soft tissue sarcoma.

Abstract

Background: Soft tissue sarcoma (STS) is a heterogeneous group of rare malignancies with limited response to conventional chemotherapy. Among these, epithelioid haemangioendothelioma (EHE) and angiosarcoma represent rare vascular sarcomas with distinct clinical behaviours, challenging treatment approaches, and poor prognoses. Doxorubicin remains the standard first-line therapy for metastatic STS, but its use is constrained by dose-dependent cardiotoxicity. Pegylated liposomal doxorubicin (PLD) has been proposed as an alternative.

Material and method: This retrospective, registry-based cohort study investigates the efficacy of PLD in patients with locally advanced or metastatic STS treated at Aarhus University Hospital, Denmark, between 2008 and 2023. Patients were identified from a regional database, and progression-free survival (PFS) and overall survival (OS) were analysed.

Results: A total of 38 patients were included, with 6 diagnosed with EHE and 16 with angiosarcoma. Among EHE patients, all had metastatic disease at diagnosis, with a median PFS of 7.8 months and OS of 1.5 years from the start of PLD treatment. Two patients remained progression-free for over 5 years. In angiosarcoma patients, the median PFS was 7.4 months, and the median OS was 2.4 years. Other STS subtype including solitary fibrous tumours (SFT), showed minimal benefit from PLD, with a median PFS of 2.8 months.

Interpretation: Pegylated liposomal doxorubicin demonstrated clinically relevant activity in angiosarcoma and EHE. It may be considered a therapeutic option for patients with these aggressive vascular sarcomas. Further prospective studies are warranted to confirm its efficacy and optimised treatment strategies.