Key result
Palliative pazopanib treatment in a 38-year-old man with primary intimal sarcoma of the heart resulted in 13 months of progression-free survival.
Why the study?
Primary intimal sarcomas of the heart are extremely rare with a dismal prognosis, complete surgical resection is only possible in 50% of patients, and management is challenging when patients are unfit for anthracycline-based therapy.
Case Report (n=1)
Pazopanib may serve as an effective first-line palliative alternative for primary intimal sarcoma of the heart when anthracycline-based chemotherapy is contraindicated, potentially offering prolonged progression-free survival.
Pazopanib may offer a tolerable option when anthracyclines are contraindicated; leaves open its role in primary cardiac intimal sarcoma.
Background: Primary intimal sarcomas of the heart are extremely rare and have a dismal prognosis. Their management represents a complex clinical challenge since complete surgical resection is the only reliable possibility of cure but is only possible in 50% of patients. In non-resectable disease, anthracycline-based therapy is the most effective treatment, but pazopanib may be used in patients unfit to receive anthracyclines. Case summary: A 38-year-old man presented with acute right heart failure symptoms due to a primary intimal sarcoma of the heart. A definite diagnosis was made after cardiac surgery. Multi-modality cardiac imaging showed early recurrence of disease with mitral valve and pulmonary veins' invasion, and the patient was deemed inoperable. Due to chronic kidney disease and previous heart failure symptoms, he was started on first-line pazopanib palliative treatment. After 11 months of chemotherapy, there was good clinical tolerance and no evidence of disease progression, which occurred after 13 months. Discussion: This case highlights the value of a multi-modality imaging approach for cardiac masses. Most importantly, it reports the successful treatment of a young patient with a primary intimal sarcoma of the heart who was started on palliative pazopanib, with a significantly higher progression-free survival than is reported in the literature. This finding may support pazopanib as a good alternative as first-line treatment when there is contraindication for anthracycline-based chemotherapy.
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Martinho et al. (2024) conducted a case report in Primary intimal sarcoma of the heart (n=1). Pazopanib was evaluated on Progression-free survival. Palliative pazopanib treatment in a 38-year-old man with primary intimal sarcoma of the heart resulted in 13 months of progression-free survival.
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