CHEST Guidelines-A-52-Year-Old-Man-With-Chest-Pain-and-Dyspnea

A-52-Year-Old-Man-With-Chest-Pain-and-Dyspnea_ches

Pdf Summary

A 52-year-old man presented with chest pain and dyspnea, initially treated with anticoagulation for a pulmonary artery filling defect detected by a CT pulmonary angiogram (CTPA) a year prior. Despite treatment with apixaban, his symptoms worsened, leading to a reevaluation revealing complete occlusion of the right pulmonary artery and pulmonary hypertension. Tests for hypercoagulable conditions revealed positive lupus anticoagulant but no antiphospholipid syndrome. Physical exams indicated right ventricular dysfunction, and repeated heart catheterization confirmed chronic thromboembolic pulmonary hypertension (CTEPH).<br /><br />The patient's persistent symptoms despite anticoagulation suggested a need for more invasive management. During pulmonary thromboendarterectomy (PTE), unexpected fibrotic tissue prompted further investigation. Histopathology of the excised tissue revealed densely packed polygonal and spindle cells, confirming a diagnosis of pulmonary artery intimal sarcoma (PAIS), a rare tumor type arising from the pulmonary artery. This entity is often mistaken for chronic thromboembolism due to overlapping radiographic features but has distinct characteristics such as entire arterial lumenal filling and vessel expansion on CTPA.<br /><br />Treatment priority for PAIS is complete surgical resection, often with adjuvant therapies like chemotherapy or radiotherapy. Following surgery, the patient underwent PET-CT scans indicating residual and metastatic disease. A chemotherapy regimen improved his condition significantly. Further molecular testing revealed high PD-L1 expression and mutations suggestive of immunotherapy responsiveness, leading to treatment with pembrolizumab. The patient showed significant improvement in symptoms and imaging results.<br /><br />The case highlights the importance of considering PAIS in patients with unresolved embolism symptoms, demonstrating the potential for successful outcomes with proper diagnosis and a multimodal treatment approach. Advances in diagnostic imaging and molecular insights offer promising pathways for improved management of this rare condition.

Keywords

pulmonary artery intimal sarcoma

chronic thromboembolic pulmonary hypertension

pulmonary thromboendarterectomy

apixaban

pulmonary artery occlusion

right ventricular dysfunction

histopathology

immunotherapy

PD-L1 expression

multimodal treatment