A 65-year-old Ukrainian female refugee with a low-differentiated adenocarcinoma of the right (R) lung (diagnosed 1.5 years earlier) reported to the Emergency Department with shortness of breath, fever, and a wet cough. A thoracotomy was performed during her previous hospitalization in Ukraine. Microscopic examination, performed at that time, confirmed low- low-differentiated G3 lung adenocarcinoma, and immunogenetic tests detected the ALK+ tyrosine kinase gene rearrangements. The patient had received several courses of treatment with ALK inhibitors — crizotinib and alectinib. Due to cancer progression, therapy with another ALK blocker, brigatinib, had been initiated in the third line. On admission to the Department of Pulmonology and Oncology, physical examination showed tachycardia, tachypnoea and a decrease in the alveolar murmur over the R lung and the upper parts of the left lung. Laboratory test results initially revealed significantly elevated markers of inflammation (leukocytosis with the left shift: leukocytes — 60750 cells/μl, neutrophils — 58660 cells/μl), elevated C-reactive protein (218 mg/l, upper limit of normal = 5 mg/l), and procalcitonin (4.72 ng/ml, upper limit of normal <5 ng/ml). Chest computed tomography angiography showed that the R lung was practically completely airless (Figure 1A) and there was a pathological nodular lesion in the R lung (adjacent to the bifurcation of the pulmonary trunk, surrounding the branches of the R pulmonary artery, infiltrating the R upper lobe artery, segmental arteries to the 3R segment and peripheral branches of the subsegmental arteries to the R middle lobe) obstructing the bronchi of the R lung (Figure 1B). We observed the impression of the nodular mass on the superior vena cava and the R atrium. A pathological soft-tissue mass was visualized in the lumen of the left atrium (LA) of approximately 63 × 32 mm (right-left × anterior-posterior), protruding through the mitral valve into the left ventricle (Figure 1B), compressing the left ventricular outflow tract (LVOT) and the aortic valve. Massive mediastinal, cervical, and supraclavicular lymphadenopathy and pathological effusion in both pleural cavities and in the pericardium were found. Right-sided pneumonia was diagnosed, secondary to the underlying disease, and broad-spectrum intravenous empirical antibiotic therapy (amoxicillin + clavulanate, ciprofloxacin) improved the patient’s condition.
Transthoracic echocardiography revealed a tumor filling almost the entire LA (Figure 1C–E), originating from the R superior pulmonary vein. Four-chamber view (color Doppler) showed stenotic mitral flow (Figure 1F). The disease was identified as T4N3M1 stage IV. The patient was disqualified from cardiac surgery by the Heart Team due to the very advanced stage of cancer. Brigatinib treatment was continued. She was discharged for further outpatient care and died a month later.
Lung adenocarcinoma with ALK gene rearrangement is a specific molecular subtype of lung adenocarcinoma [1] characterized by a high ability to give rise to distant metastases, including heart cavities. Mechanisms by which LA involvement occurs may be direct invasion by the primary tumor, involvement of lymph nodes, or, the least common, pulmonary venous transfer of the original lesion [2]. The condition requires surgery, which, however, may involve the risk of neoplastic dissemination, hemorrhage, and a higher probability of infection [3, 4]. The decision on the best therapeutic approach should be made by a multidisciplinary team, regarding especially life expectancy. Lung cancer resection can be performed simultaneously with cardiac surgery, but in advanced cases, heart surgery takes precedence over that of the lung [5]. If cardiac surgery is not possible, conservative preparatory treatment with highly selective ALK inhibitors such as alectinib, crizotinib, and brigatinib is recommended.
Article information
Conflict of interest: None declared.
Funding: None.
Open access: This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, which allows downloading and sharing articles with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially. For commercial use, please contact the journal office at kardiologiapolska@ptkardio.pl.
