Key result
Pazopanib yields ~40% disease stabilization in unresectable primary cardiovascular sarcoma.
Why the study?
Primary cardiovascular sarcoma is a rare, aggressive malignancy with poor prognosis and limited treatment options for unresectable disease.
Observational (n=5)
No
Pazopanib may offer meaningful disease control and long-term stabilization for selected patients with unresectable primary cardiovascular sarcoma, provided treatment-related toxicities are proactively managed.
Primary cardiovascular sarcoma (PCS) is a rare, aggressive malignancy with a poor prognosis. The present study describes a single-center experience with pazopanib, an oral multikinase inhibitor, for the outpatient management of unresectable PCS. A retrospective review was conducted of consecutive patients with sarcoma involving the heart and/or pulmonary artery treated with pazopanib at Ehime University Hospital (Toon, Japan) between January 2015 and December 2025. Pazopanib was initiated at 200-400 mg daily and escalated to 600-800 mg according to tolerability. Five patients aged 56-81 years were identified, with angiosarcoma (n=1), intimal sarcoma (n=2) or leiomyosarcoma (n=2). Two patients achieved durable stable disease for >500 days. A case of right atrial angiosarcoma with lung metastasis remained stable for 33 months when treated with 800 mg pazopanib; the patient later died of recurrent cerebral infarction. A case of pulmonary artery intimal sarcoma remained stable for 22 months when treated with 400-600 mg pazopanib; however, treatment was discontinued because of fatigue. Two additional patients stopped treatment because dose escalation led to intolerable toxicities. One patient continued therapy with 800 mg pazopanib as of 8 months, with stable disease, despite requiring a pericardial window for malignant pericardial effusion during treatment. In conclusion, pazopanib can provide meaningful disease control and long-term stabilization for selected patients with unresectable PCS. As treatment-related toxicities frequently limit therapy, individualized dose management and proactive supportive care are essential to maintain treatment continuity and to maximize clinical benefit.
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Yamanaka et al. (2026) conducted an observational in Primary cardiovascular sarcoma (n=5). Pazopanib was evaluated on Disease control / stable disease. In a retrospective series of 5 patients with unresectable primary cardiovascular sarcoma, pazopanib exposure provided durable disease stabilization for over 500 days in two patients.
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