Key result
A 70-year-old male presenting with progressive dyspnea was diagnosed with a rapidly growing primary pulmonary leiomyosarcoma originating from the pulmonary artery that invaded the right atrium and neighboring structures.
Case Report (n=1)
This case highlights the diagnostic challenge of primary pulmonary artery leiomyosarcoma and the utility of bedside cardiopulmonary ultrasound in evaluating severe dyspnea.
May warrant sarcoma consideration in unexplained dyspnea; leaves open optimal diagnostic pathways for this rare entity.
Leiomyosarcomas are rare neoplasms of the smooth muscles. Primary pulmonary leiomyosarcomas, which constitute approximately 0.2%-0.5% of all primary lung malignancies, are extremely rare and highly lethal. They may originate from the smooth muscle cells of the bronchial wall, the blood vessels, or the pulmonary interstitium, and their rare occurrence, localization, and nonspecific clinical symptoms mean that correct diagnosis and proper management are often delayed. Here, we report a rapidly growing primary pulmonary leiomyosarcoma, which invaded the right atrium, vena cava superior, mediastinum, right hilar area, and left pulmonary artery within 4 months. On histopathology, a transthoracic needle biopsy of the mass confirmed leiomyosarcoma, and delayed presentation meant that there was a local spread to the neighboring structures at the time of diagnosis.
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Demirci et al. (2018) conducted a case report in Pulmonary artery leiomyosarcoma (n=1). Diagnostic workup was evaluated. A 70-year-old male presenting with progressive dyspnea was diagnosed with a rapidly growing primary pulmonary leiomyosarcoma originating from the pulmonary artery that invaded the right atrium and neighboring structures.
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