What is the clinical evidence from this study?

Study design: Cohort. Population: Locally advanced or metastatic soft tissue sarcoma (n=38). Intervention: Pegylated liposomal doxorubicin. Primary outcome: Progression-free survival (PFS).

What does this research mean for the field?

Pegylated liposomal doxorubicin demonstrates clinically relevant activity and provides progression-free survival benefits in patients with aggressive vascular sarcomas such as angiosarcoma and epithelioid haemangioendothelioma, but shows minimal benefit in other soft tissue sarcoma subtypes. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

April 23, 2025Open Access

The use of pegylated liposomal doxorubicin in metastatic soft tissue sarcoma

Key Result

Pegylated liposomal doxorubicin demonstrated clinically relevant activity in angiosarcoma and epithelioid haemangioendothelioma, with a median progression-free survival of 7.4 and 7.8 months, respectively.

Study Design

Type

Cohort (n=38)

Multicenter

Structured PICO

Does pegylated liposomal doxorubicin improve progression-free survival and overall survival in patients with locally advanced or metastatic soft tissue sarcoma?

Population

38 adults (median age 64, 47% male) with locally advanced or metastatic soft tissue sarcoma (STS), including 6 with epithelioid haemangioendothelioma (EHE) and 16 with angiosarcoma, treated at Aarhus University Hospital, Denmark, between 2008 and 2023.

Intervention

Pegylated liposomal doxorubicin (PLD)

Comparator

Historical reference groups from the Danish Sarcoma Database with histologically confirmed EHE, angiosarcoma, and solitary fibrous tumours (SFT) not treated with PLD

Outcome

Progression-free survival (PFS), defined as time from the first PLD treatment until progression of the disease (start of another treatment or death)hard clinical

Pegylated liposomal doxorubicin demonstrated clinically relevant activity in angiosarcoma and EHE, suggesting it as a viable therapeutic option for these aggressive vascular sarcomas.

Limitations

Low number of patients treated
Retrospective study design

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.

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