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      Insights on pulmonary tumor thrombotic microangiopathy: a seven-patient case series

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          Abstract

          Pulmonary tumor thrombotic microangiopathy (PTTM) is a disease process wherein tumor cells are thought to embolize to the pulmonary circulation causing pulmonary hypertension (PH) and death from right heart failure. Presented herein are clinical, laboratory, radiographic, and histologic features across seven cases of PTTM. Highlighted in this publication are also involvement of pulmonary venules and clinical features distinguishing PTTM from clinical mimics.

          We conducted a retrospective chart review of seven cases of PTTM from hospitals in the greater Los Angeles metropolitan area.

          Patients in this series exhibited: symptoms of cough and progressive dyspnea; PH and/or heart failure on physical exam; laboratory abnormalities of anemia, thrombocytopenia, elevated LDH, and elevated D-dimer; chest computed tomography (CT) showing diffuse septal thickening, mediastinal and hilar lymphadenopathy and nodules; elevated pulmonary artery pressures on transthoracic echocardiogram and/or right heart catheterization; and presence of malignancy. Tumor emboli and fibrocellular intimal proliferation were seen in pulmonary arterioles, while two patients had pulmonary venopathy.

          PTTM is a devastating disease occurring in patients with metastatic carcinoma. An early diagnosis is challenging. Understanding the clinical presentation of PTTM and distinguishing PTTM from clinical mimics may help achieve an early diagnosis and allow time for initiation of treatment.

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          Pulmonary tumor thrombotic microangiopathy with pulmonary hypertension.

          A Illes, H Otto, Mark A. Stein … (1990)
          Pulmonary tumor thrombotic microangiopathy is characterized by fibrocellular intimal proliferation of small pulmonary arteries and arterioles in patients with metastatic carcinoma. Its morphologic features, including precursor lesions, were studied in 21 patients diagnosed in 630 consecutive autopsy cases with carcinoma (3.3%). Nineteen of 21 patients had adenocarcinoma and 11 of these 19 patients had gastric carcinoma. The pathogenetic events start with microscopic tumor cell embolism. Tumor emboli do not occlude affected vessels but induce both local activation of coagulation and fibrocellular intimal proliferation, which lead into stenosis or occlusion. Hemodynamically, an increase in vascular resistance results in pulmonary hypertension. In three patients, metastatic carcinoma was unknown before death, and the condition was diagnosed as pulmonary hypertension of unknown origin. Thus, pulmonary tumor thrombotic microangiopathy should be considered in the differential diagnosis of primary pulmonary hypertension, particularly in patients with well-known carcinoma who develop acute or subacute cor pulmonale.
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            The Pathobiology of Chronic Thromboembolic Pulmonary Hypertension.

            Anton Vonk Noordegraaf, Peter Dorfmüller, Irene Lang (2016)
            Chronic thromboembolic pulmonary hypertension (CTEPH) is a late sequel of venous thromboembolism that cannot be completely reproduced in animal models. The prevalence of CTEPH in humans is estimated at roughly 17-20 per million; however, partly because up to 50% of patients with CTEPH never experience symptomatic pulmonary embolism, precise numbers on the incidence and prevalence are not known. Because CTEPH is diagnosed at a median age of 63 years in patients who often have other concomitant cardiovascular disease or lung disease, assessment of pathophysiology in patients can be challenging, We do know that CTEPH is a dual vascular disorder. Stenoses, webs, and occlusions predominate in large and medium-sized pulmonary arteries at the sites of previous pulmonary emboli. A "secondary vasculopathy" resembling the pulmonary arteriopathy encountered in other forms of pulmonary hypertension predominates in low-resistance vessels. Anastomoses between bronchial artery branches and precapillary pulmonary arterioles appear during evolution of the disease. Other acquired vascular connections between bronchial arteries and pulmonary veins may trigger venous remodeling. Current concepts regarding the pathophysiology of CTEPH include contributions of hyperactive coagulation (e.g., high coagulation factor VIII, combined coagulation defects, dysfibrinogenemias), insufficient anticoagulation, non-O blood groups, and misguided thrombus resolution (e.g., infection, inflammation, dysfunctional innate immunity, abnormal circulating phospholipids). Current research focuses on the question as to whether a genetic predisposition leads to misguided vascular healing after pulmonary thromboembolism in susceptible individuals.
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              CT diagnosis of chronic pulmonary thromboembolism.

              Eva Castañer, Xavier Gallardo, Eva Ballesteros … (2015)
              Chronic pulmonary thromboembolism is mainly a consequence of incomplete resolution of pulmonary thromboembolism. Increased vascular resistance due to obstruction of the vascular bed leads to pulmonary hypertension. Chronic thromboembolic pulmonary hypertension is clearly more common than previously was thought, and misdiagnosis is common because patients often present with nonspecific symptoms related to pulmonary hypertension. Computed tomography (CT) is a useful alternative to conventional angiography not only for diagnosing chronic pulmonary thromboembolism but also for determining which cases are treatable with surgery and confirming technical success postoperatively. The vascular CT signs include direct pulmonary artery signs (complete obstruction, partial obstruction, eccentric thrombus, calcified thrombus, bands, webs, poststenotic dilatation), signs related to pulmonary hypertension (enlargement of main pulmonary arteries, atherosclerotic calcification, tortuous vessels, right ventricular enlargement, hypertrophy), and signs of systemic collateral supply (enlargement of bronchial and nonbronchial systemic arteries). The parenchymal signs include scars, a mosaic perfusion pattern, focal ground-glass opacities, and bronchial anomalies. The presence of one or more of these radiologic signs arouses suspicion and allows diagnosis of this entity. Early recognition of chronic pulmonary thromboembolism may help improve the outcome, since the condition is potentially curable with pulmonary thromboendarterectomy. (c) RSNA, 2009.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Pulm Circ
                Journal ID (iso-abbrev): Pulm Circ
                Journal ID (publisher-id): PUL
                Journal ID (hwp): sppul
                Title: Pulmonary Circulation
                Publisher: SAGE Publications (Sage UK: London, England )
                ISSN (Print): 2045-8932
                ISSN (Electronic): 2045-8940
                Publication date (Electronic): 25 August 2017
                Publication date Collection: December 2017
                Volume: 7
                Issue: 4
                Pages: 813-820
                Affiliations
                [1 ]Division of Pulmonary and Critical Care Medicine, University of California, Irvine, CA, USA
                [2 ]Division of Pulmonary and Critical Care Medicine, University of California, Los Angeles David Geffen School of Medicine, Los Angeles, CA, USA
                [3 ]Division of Pulmonary and Critical Care Medicine, West Los Angeles Veterans Affairs Medical Center, Los Angeles, CA, USA
                [4 ]Department of Pathology and Laboratory Medicine, University of California, Los Angeles David Geffen School of Medicine, Los Angeles, CA, USA
                [5 ]Department of Radiological Sciences, University of California, Los Angeles David Geffen School of Medicine, Los Angeles, CA, USA
                [6 ]Division of Pulmonary and Critical Care Medicine, Olive View-UCLA Medical Center
                Author notes
                [*]Nader Kamangar, Chief, Division of Pulmonary & Critical Care Medicine, Vice Chair, Department of Medicine, Olive View-UCLA Medical Center, Professor of Medicine, David Geffen School of Medicine at UCLA, 14445 Olive View Drive, Room 2B-182, Sylmar, CA 91342, USA. Email: kamangar@ 123456ucla.edu
                Author information
                http://orcid.org/0000-0002-1535-7315
                Article
                Publisher ID: 10.1177_2045893217728072
                DOI: 10.1177/2045893217728072
                PMC ID: 5703123
                PubMed ID: 28782988
                SO-VID: ea902858-034e-46a3-a114-2fe5f29d72aa
                Copyright © © The Author(s) 2017
                License:

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                Date received : 20 April 2017
                Date accepted : 3 August 2017
                Categories
                Subject: Case Reports
                Custom metadata
                cover-date October-December 2017

                ScienceOpen disciplines: Respiratory medicine
                Keywords: pulmonary pathology,chest imaging,pulmonary arterial hypertension
                Data availability:
                ScienceOpen disciplines: Respiratory medicine
                Keywords: pulmonary pathology, chest imaging, pulmonary arterial hypertension

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