Metastatic Non-Small-Cell Lung Cancer in the Setting ofUnilateral Agenesis of the Left Pulmonary Artery: A CaseReport and Comprehensive Literature Review

Unilateral absence of the pulmonary artery (UAPA) represents a rare condition that is often associated with cardiac congenitalabnormalities but can also be relatively asymptomatic and indolent. There is a lack of consensus regarding the management ofUAPA. However, in the setting of associated complications and ongoing infection, pulmonary resection is advocated. Althoughrare, the association between UAPA and bronchogenic carcinoma has been previously reported in seven published cases. Inthe majority of these, anatomic lung resection (most commonly with pneumonectomy) was curative. We present thefirstreported case of ipsilateral metastatic non-small-cell lung cancer- (NSCLC-) associated UAPA in a 47-year-old patient withventilator-dependent hypoxic respiratory failure and bronchorrhea, who had been lost to follow-up for 8 years. Initialinvestigations did not yield evidence of malignancy, and confirmation of metastatic disease was made intraoperatively at thetime of thoracotomy. Thefindings demonstrated evidence of diffuse metastatic pleural disease with lymphangiticcarcinomatosis and superimposed infection. The patient was palliated and passed away shortly thereafter. In the setting ofUAPA, clinicians should have a high index of suspicion for the possibility of malignancy, and if proven, they should considerearly resection following appropriate staging.