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Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Epidemiology, etiology, and pathogenesis

Author
Gerald L Weinhouse, MD
Section Editor
Jess Mandel, MD
Deputy Editor
Geraldine Finlay, MD

INTRODUCTION

The lungs are a prominent target for the embolization of any material larger than approximately 10 microns that gains access to the venous or lymphatic circulation. This includes venous thrombi, air, amniotic fluid, fat, injected foreign material, and tumor. The malignant pulmonary embolization syndromes, pulmonary tumor emboli and lymphangitic carcinomatosis, are associated with a poor prognosis. Thus, prompt recognition is critical so that therapy can be initiated in a timely fashion.

The epidemiology, etiology, and pathogenesis of pulmonary tumor emboli and pulmonary lymphangitic carcinomatosis are reviewed here. The clinical features, diagnostic evaluation, and management of pulmonary tumor emboli and pulmonary lymphangitic carcinomatosis and the embolization of venous thrombi, air, amniotic fluid, fat, or foreign material are discussed separately. (See "Overview of acute pulmonary embolism in adults" and "Air embolism" and "Amniotic fluid embolism syndrome" and "Fat embolism syndrome" and "Foreign body granulomatosis" and "Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Diagnostic evaluation and management".)

TERMINOLOGY

Pulmonary tumor embolism and lymphangitic carcinomatosis are considered end-stage manifestations of malignancy. The terminology for both disorders is often used interchangeably and while they are often considered as morphologically distinct from each other, overlap is not infrequent [1]. Both disorders need to be distinguished from pulmonary metastases, which are identified pathologically as solid isolated lesions that have the classic morphologic features of lung invasion. (See "Overview of the risk factors, pathology, and clinical manifestations of lung cancer", section on 'Clinical manifestations'.)  

Pulmonary tumor embolism — Pulmonary tumor embolism refers to the identification of tumor within pulmonary blood vessels on pathologic lung samples. Invasion of the surrounding interstitium is not typically seen, a feature that distinguishes it from pulmonary metastases. However, tumor may sometimes also be concomitantly seen within lymphatic vessels. Due to the prominent involvement of pulmonary vasculature, patients with this syndrome are more likely to present with the signs and symptoms of pulmonary hypertension and acute right heart failure. (See "Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Diagnostic evaluation and management", section on 'Clinical presentation'.)

Pulmonary lymphangitic carcinomatosis — Lymphangitic carcinomatosis is morphologically defined by the presence of tumor within pulmonary lymphatics. Tumor cells within pulmonary veins are not typically seen; however, tumor cells can invade the interstitium resulting in thickened bronchovascular bundles and interstitial septae. Since pulmonary capillary obstruction is not a feature of this disorder, patients with this syndrome are less likely to present with the signs and symptoms of pulmonary hypertension and acute right heart failure. (See "Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Diagnostic evaluation and management", section on 'Clinical presentation'.)

      

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Literature review current through: Nov 2016. | This topic last updated: Wed Apr 06 00:00:00 GMT 2016.
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