Pulmonary artery intimal sarcoma in TB-endemic region: A diagnostic challenge

Abstract

Pulmonary artery intimal sarcoma (PAIS) is a rare, aggressive mesenchymal tumor arising in the large vessels of pulmonary circulation with nonspecific clinical features that can resemble common pulmonary conditions such as tuberculosis (TB), particularly in TB-endemic regions. We report a 51-year-old woman with prior TB exposure who presented with a two-month history of productive cough, dyspnea, low-grade fever, and weight loss. Chest radiography showed multifocal patchy opacities and right pleural effusion, leading to empirical treatment for smear-negative TB. Her condition deteriorated, and contrast-enhanced CT revealed a lobulated mass occupying the pulmonary arteries with pulmonary and pleural metastases. Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) with histopathological analysis confirmed PAIS. Despite palliative management, including bronchial artery embolization for hemoptysis, the disease progressed rapidly, and the patient died three months after diagnosis. This case highlights the diagnostic challenges of PAIS and the importance of maintaining a broad differential diagnosis. Early recognition is essential due to the tumor's aggressive progression and poor prognosis.