SESSION TITLE: Pulmonary Vascular Disease Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 26, 2015 at 11:00 AM - 12:00 PM
INTRODUCTION: Pulmonary tumor thrombotic microangiopathy (PTTM) manifests as rapidly evolving respiratory failure and is rarely diagnosed pre-mortem.
CASE PRESENTATION: A 46 year old male was evaluated for new onset hypoxia while hospitalized for obstructive jaundice. One week prior, he was diagnosed with gastric adenocarcinoma after presenting with early satiety. He was afebrile with minimal dyspnea. A CT pulmonary angiogram was negative for PE, but demonstrated diffuse bilateral tree-in-bud and irregular nodular opacities, mediastinal and hilar adenopathy, and tiny bilateral effusions. Bronchoscopy revealed vascular prominence in the right middle lobe. EBUS-guided needle aspiration of station 10R confirmed metastatic adenocarcinoma. He was dismissed on 3L/minute O2. Five days later, his oxygen saturation was 80% in the oncology clinic, along with progressive dyspnea. Chemotherapy had not yet been started. He was admitted to the ICU and required 60% FiO2 via high flow nasal cannula. A CT pulmonary angiogram was again negative for PE, but demonstrated progression of the diffuse nodular infiltrates and bilateral effusions (Image). Echocardiogram, normal 5 days earlier, was now notable for RVSP of 75 mmHg, RV enlargement and mildly reduced systolic function. He was started on chemotherapy, dexamethasone, and unfractionated heparin, and was dismissed home 8 days later on 2L/minute O2, low molecular weight heparin, and palliative chemotherapy. He passed away 3 months after his initial presentation.
DISCUSSION: PTTM presents a challenging and ominous scenario. Tumor cells in the pulmonary vasculature lead to occlusion of small vessels, activation of the coagulation cascade, and intimal proliferation thought to be mediated by VEGF. Due to the rapid progression of pulmonary hypertension and right heart failure, most cases of PTTM are diagnosed post-mortem. In patients presenting earlier, typically with dyspnea and hypoxia, this can be the initial manifestation of malignancy, most commonly adenocarcinoma, including breast, pancreatic, and gastric. Imaging is notable for diffuse micronodular and irregular infiltrates, with evidence of elevated right-sided pressures. Limited evidence is available regarding management.
CONCLUSIONS: PTTM should be considered in unexplained rapidly progressive respiratory failure, even without a known diagnosis of malignancy.
Reference #1: Chinen K, et al. PTTM in patients with gastric carcinoma: an analysis of 6 autopsy cases and review of the literature. Pathology - Research and Practice 2010;206:682-689.
DISCLOSURE: The following authors have nothing to disclose: Lynn Fussner, Eva Carmona
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