Pulmonary Artery Sarcoma Diagnosis and Management

Pulmonary artery sarcoma is a rare, highly aggressive malignancy originating in the intimal layer of the pulmonary vasculature, frequently masquerading as chronic thromboembolic disease. Clinical features—such as progressive dyspnoea, chest pain and right ventricular dysfunction—often prompt initial anticoagulant or thrombolytic therapy, delaying definitive treatment. Accurate diagnosis combines imaging modalities, including contrast-enhanced CT angiography and PET, with histopathology obtained by surgical or endovascular biopsy. Management centres on radical surgical resection, typically via pulmonary endarterectomy under cardiopulmonary bypass, supplemented by tailored chemotherapy and radiotherapy. Advances in molecular profiling revealing oncogenic amplifications in MDM2, CDK4 and PDGFRA have begun to inform targeted therapies, underscoring the need for multidisciplinary care to improve survival in this globally significant disease.

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Pulmonary Artery Sarcoma Diagnosis and Management publication trend

The graph below shows the total number of articles in pulmonary artery sarcoma diagnosis and management across all publications each year (not limited to Nature Index journals).

Technical terms

Pulmonary endarterectomy (PEA): Surgical removal of obstructive intraluminal material from pulmonary arteries under cardiopulmonary bypass to restore blood flow.

Pulmonary artery computed tomography angiography (PACTA): High-resolution, contrast-enhanced CT imaging technique visualising intravascular anatomy and filling defects.

Wall eclipsing sign: Imaging feature where a sarcomatous lesion envelops and distends the arterial wall, distinguishing tumour from thrombus.

Intimal sarcoma: Malignant mesenchymal tumour arising from the innermost layer of large blood vessels, notably the pulmonary artery.

Endovascular catheter biopsy: Minimally invasive procedure to obtain intravascular tissue samples using aspiration or forceps under imaging guidance.

References

1. Intimal Sarcoma with MDM2/CDK4 Amplification and p16 Overexpression: A Review of Histological Features in Primary Tumor and Xenograft, with Immunophenotype and Molecular Profiling. International Journal of Molecular Sciences (2023).
2. The Wall Eclipsing Sign on Pulmonary Artery Computed Tomography Angiography Is Pathognomonic for Pulmonary Artery Sarcoma. PLOS ONE (2013).
3. Multimodal Approach of Pulmonary Artery Intimal Sarcoma: A Single‐Institution Experience. Sarcoma (2017).
4. Unique Aberrations in Intimal Sarcoma Identified by Next-Generation Sequencing as Potential Therapy Targets. Cancers (2019).
5. Endovascular Catheter Biopsy for the Diagnosis of Pulmonary Artery Sarcoma. Annals of Vascular Diseases (2019).