A case of recurrent pulmonary artery intimal sarcoma after left pneumonectomy

Background: Pulmonary intimal sarcoma is a rare sarcoma arising from the pluripotent intimal cells of the pulmonary artery. We report a patient with a pulmonary mass who was initially diagnosed with nonsmall cell lung cancer (NSCLC). An intimal sarcoma was intraoperatively revealed in the left main pulmonary artery and her clinical course was severe. Case: A 54-year-old woman presented with left back pain. Computed tomography (CT) showed a 5 cm mass in the lower lobe of the left lung. The initial diagnosis, based on a CT-guided core-needle lung biopsy specimen, was NSCLC. She was referred to our hospital. Contrast-enhanced CT showed a vague low-density area in the left main pulmonary artery. However, it was regarded as an artifact because of the lack of reproducibility. Reevaluation, including an immunohistochemical analysis of the sample (which was obtained at the previous hospital), revealed that the tumor was a sarcoma. Although a left lower lobectomy was attempted, the tumor in the left main pulmonary artery was intraoperatively palpated and it corresponded to the filling defect on CT. Therefore, the tumor and the mass in the lower lobe were diagnosed as the primary intimal sarcoma and its metastasis, respectively. Pneumonectomy was performed. Recurrence occurred in the pulmonary artery one month later. Second surgery on extracorporeal cardiopulmonary bypass was performed and the patient was disease-free at 4 months; however, he died of the disease 3.5 months later. Conclusion: Clinicians should maintain suspicion of intimal sarcoma in patients with a filling defect in the pulmonary artery, even when a solitary pulmonary mass is detected.