article · Egyptian Journal of Bronchology
Pulmonary artery intimal sarcoma is a rare and aggressive malignancy originating in the intimal layer of the pulmonary artery. Because its symptoms and imaging features resemble thromboembolic conditions, it frequently leads to delayed identification. In an illustrative clinical case, a 38-year-old male presenting with chest pain, hypoxemia, and progressive breathlessness was initially treated with anticoagulants for presumed pulmonary embolism. When deterioration continued, chronic thromboembolic pulmonary hypertension was suspected, leading to pulmonary endarterectomy. During surgery, clinicians discovered a mass-like lesion rather than a blood clot. Subsequent histopathological analysis and immunohistochemistry confirmed a high-grade spindle cell sarcoma positive for MDM2 and CDK4. Positron emission tomography revealed metabolically active lesions across the pulmonary artery, peri-pulmonary regions, and mediastinum, resulting in multidisciplinary oncological management with gemcitabine and docetaxel chemotherapy.
Distinguishing rare vascular malignancies from common thromboembolic diseases remains a serious diagnostic challenge. When patients fail to improve under standard anticoagulant therapy, recognising the features of pulmonary artery intimal sarcoma prevents prolonged misdiagnosis. Early detection through comprehensive diagnostic workups ensures patients receive appropriate surgical interventions and multidisciplinary oncological treatment rather than ineffective therapies for chronic blood clots.
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Abstract Background Pulmonary artery intimal sarcoma (PAIS) is an exceptionally rare and aggressive mesenchymal malignancy arising from the intimal layer of the pulmonary artery. Owing to its nonspecific clinical manifestations and radiologic resemblance to pulmonary thromboembolic disease, delayed diagnosis is common. Case presentation We report the case of a 38-year-old male who presented with progressive dyspnea, pleuritic chest pain, and hypoxemia. Computed tomography pulmonary angiography initially demonstrated bilateral saddle-shaped pulmonary arterial filling defects, and the patient was treated for presumed acute pulmonary embolism with anticoagulation. Despite adequate anticoagulation, clinical and radiological deterioration occurred, prompting subsequent evaluation for presumed chronic thromboembolic pulmonary hypertension (CTEPH). Echocardiography revealed pulmonary hypertension with preserved ventricular function. The patient underwent pulmonary endarterectomy for presumed operable CTEPH. Intraoperatively, the obstructive lesion appeared mass-like rather than thrombotic. Histopathological examination demonstrated a high-grade spindle cell sarcoma. Immunohistochemistry demonstrated positivity for MDM2 and CDK4 and negativity for SMA, CD31, and S100, supporting the diagnosis of pulmonary artery intimal sarcoma. Subsequent 18 F-FDG PET/CT demonstrated metabolically active lesions involving the pulmonary artery and its branches, with additional peri-pulmonary and mediastinal lesions. The patient was referred for multidisciplinary oncologic management and started on gemcitabine plus docetaxel chemotherapy. Conclusion PAIS is a rare but important differential diagnosis of pulmonary embolism and presumed CTEPH, particularly in patients with progressive symptoms or persistent pulmonary arterial filling defects despite adequate anticoagulation. Early recognition and multimodal evaluation may facilitate appropriate diagnosis and multidisciplinary management.
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DOI: 10.1186/s43168-026-00654-9
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