A 47-year-old HIV-negative female from northeast Africa was initially seen in consultation by the pulmonology service in 2007 for evaluation of positive TB skin test, abnormal chest X-ray (CXR) findings, and increasing symptoms of productive cough. She was a life-long nonsmoker, and her past medical history was significant for long-standing hypertension, osteoarthritis of the knee, and obesity. At the time, CXR demonstrated a density in the posterior left lower lobe and a poorly defined left hemidiaphragm. Bronchoscopy showed no obvious endobronchial abnormalities noted. Washings from the left upper and lower lobes consisted of negative cultures and negative acid-fast bacilli (AFB) analysis. Cytological analysis yielded a few multinucleated giant cells. CT of the chest demonstrated an enlarged right pulmonary artery and a slightly enlarged right atrium, with associated radiographic abnormalities of the left lung, including multifocal areas of consolidation, subpleural ground glass opacities and reticulation, and bronchiectasis. More notably, there was evidence of hypertrophied bronchial arteries providing collateral circulation to the left lung (Figures 1 and 2).
The patient was lost to follow-up and seen again in 2015, at the age of 55, for evaluation of chronic pulmonary infiltrates, accompanied by symptoms of fever, cough, copious watery sputum production, low-volume hemoptysis, decreased exercise tolerance, and left-sided chest pain. Repeat CT scan examination confirmed absence of the left pulmonary artery with near complete consolidation of the left lung, as well as left-sided mediastinal shift and a small left pleural effusion. The right lung contained multifocal nodular opacities in all lobes (Figures 3 and 4). Given the radiographic and clinical changes, the patient was thought to have a primary infectious process (likely MTB complex) and was placed on respiratory isolation. Repeat bronchoscopy was performed with no growth of tuberculosis or other infectious agent and persistently negative AFB. The presumed diagnosis at that time was infectious vs. inflammatory in nature, with a remote possibility of malignancy still being considered. A thoracic surgical consultation was sought out, for consideration of open lung biopsy as a means of ruling out malignancy and to consider left pneumonectomy for symptom control.
The patient's symptoms ultimately continued to increase, requiring admission to a hospital for supplemental oxygen delivery. Transthoracic and surgical biopsies were deemed to be too highly risky and were ultimately not pursued. Despite aggressive medical therapy and broad spectrum antibiotic coverage, she required transfer to the intensive care unit due to hypoxic respiratory failure. Multidisciplinary conferencing concluded that the necrotic left lung was the primary culprit in the patient's decline, with acute chronic infection likely related to right lung contamination being the most likely diagnosis. The patient continued to decline clinically. She developed multisystem organ failure including respiratory compromise leading to intubation and mechanical ventilation, hypotension requiring ionotropic support, and worsening renal function. Accordingly, the decisive treatment option was for an emergent high-risk left pneumonectomy with consideration of preoperative embolization of collateral hypertrophied bronchial vessels. Preoperative CT investigation demonstrated a consolidated necrotic left lung, with moderate left pleural effusion and areas of thickening of the left pleura. On cardiopulmonary testing, echocardiogram revealed left-sided ejection fraction of 65-70%, with preserved right ventricular systolic function, without any significant tricuspid regurgitation. Quantitative lung ventilation-perfusion scanning identified 1% ventilation and perfusion to the left lung, with >90% right-sided lung perfusion. The patient and her family were quoted a near 50-90% surgical mortality, in the face of imminent death without intervention.
The patient was taken to the operating room and placed in the right lateral decubitus position, and an endobronchial blocker was placed through her single-lumen endotracheal tube and positioned in the left mainstem bronchus. A posterolateral thoracotomy was performed, after harvesting a serratus anterior muscle flap. Upon entry into the pleural space, it was evident that the left lung was cemented to the parietal pleura with dense thick adhesions. Minimal dissection was undertaken in order to identify an area of the pleural cavity that was free. Five hundred milliliters of serous pleural fluid was drained. Additionally, there was evidence of multiple small pleural nodules dispersed throughout the chest cavity and the hilum. Frozen section analysis of these pleural deposits confirmed the presence of metastatic adenocarcinoma, confirming the presence of stage IV lung cancer. As such, the planned left pneumonectomy was aborted and the chest was closed with an indwelling chest tube.
She was transferred back to the ICU with continued mechanical ventilation. The working diagnosis at that point was diffuse metastatic lung cancer with lymphangitic carcinomatosis and superimposed infection. After the family was informed of the operative findings, a palliative approach was undertaken. Comfort measures were ensured. Mechanical ventilation was weaned off as care was being withdrawn, and the patient peacefully passed away.
Pleural fluid analysis identified the presence of malignant cells suggestive of adenocarcinoma. Final pathologic analysis of intraoperative specimens demonstrated pleural tissue comprised of tall columnar tumor cells with focal necrosis, infiltrated by adenocarcinoma (thyroid transcription factor-1 positive) with predominantly papillary features. Epidermal growth factor receptor (EGFR) mutation was not identified (wild-type allele) by polymerase chain reaction (PCR) analysis using the EntroGen EGFR Kit Exons 18, 19, 20, and 21. In addition, ALK rearrangement was not identified.