A 65-year-old woman with rapidly progressive pulmonary hypertension was found to have malignant pulmonary artery obstruction with sarcomatous histology, mimicking acute pulmonary thromboembolism.
Case Report (n=1)
No
Malignant pulmonary artery invasion is a critical non-thrombotic cause of pulmonary hypertension that can mimic acute pulmonary thromboembolism, necessitating multimodality imaging for accurate diagnosis.
Pulmonary hypertension (PH) caused by malignant obstruction of the pulmonary arteries is rare and may closely mimic acute pulmonary thromboembolism (PTE), particularly in patients with underlying malignancy. We report a case of rapidly progressive PH due to tumor-related pulmonary artery (PA) obstruction with sarcomatous histology. A 65-year-old woman presented with an intraluminal PA lesion initially interpreted as acute PTE. However, intense fluorodeoxyglucose uptake on positron emission tomography-computed tomography and lack of response to anticoagulation strongly suggested malignant invasion. Despite systemic chemotherapy, the tumor progressed, resulting in rapid hemodynamic deterioration with severe PH, right ventricular dysfunction on echocardiography. Although a definitive diagnosis of pulmonary artery intimal sarcoma (PAIS) could not be established, the clinical course and imaging findings were consistent with a PAIS-like phenotype. This case highlights malignant PA invasion as an important non-thrombotic cause of PH that may be easily mistaken for PTE and emphasizes the critical role of multimodality imaging in early recognition.
Kim et al. (2026) conducted a case report in Pulmonary hypertension due to malignant pulmonary artery obstruction (n=1). Systemic chemotherapy and anticoagulation was evaluated. A 65-year-old woman with rapidly progressive pulmonary hypertension was found to have malignant pulmonary artery obstruction with sarcomatous histology, mimicking acute pulmonary thromboembolism.