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Updated: Jun 4, 2026

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Recurrent pulmonary intimal sarcoma involving the right ventricular outflow tract
Dipesh K Shah1, Lyle D Joyce, Martha Grogan
1Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA.
Intimal sarcoma of the pulmonary artery, a rare cancer, is often misdiagnosed. Surgical treatment followed by chemoradiation therapy showed tumor regression in a patient with advanced disease.
Area of Science:
- Cardiovascular Pathology
- Surgical Oncology
- Medical Oncology
Background:
- Intimal sarcoma of the pulmonary artery is a rare malignancy.
- It is frequently misdiagnosed as chronic pulmonary embolism.
- The tumor can involve the right ventricular outflow tract and pulmonary valve.
Observation:
- A patient with intimal sarcoma of the pulmonary artery involving the right ventricular outflow tract and pulmonary valve underwent surgical treatment.
- The sarcoma recurred approximately 8 weeks after surgery.
- The patient received chemoradiation therapy for the recurrent tumor.
Findings:
- The recurrent intimal sarcoma showed a favorable response to chemoradiation therapy.
- Signs of tumor regression were observed following treatment.
- This case highlights the potential of multimodal therapy in managing advanced pulmonary artery sarcoma.
Implications:
- Accurate and timely diagnosis of pulmonary artery intimal sarcoma is crucial.
- Multimodal treatment including surgery and chemoradiation may improve outcomes.
- Further research is needed to establish optimal treatment strategies for this rare cancer.
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