Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Diagnostic evaluation and management
- Gerald L Weinhouse, MD
Gerald L Weinhouse, MD
- Assistant Professor of Medicine
- Harvard Medical School
Pulmonary tumor emboli and lymphangitic carcinomatosis are rare, end-stage, manifestations of malignancy that have a poor prognosis. Recognition of either of these problems should lead to a frank discussion of treatment options and/or re-evaluation of goals of care.
The clinical features, diagnosis, and treatment of pulmonary tumor embolism and pulmonary lymphangitic carcinomatosis are reviewed here. The epidemiology, etiology, and pathogenesis of these syndromes as well as the diagnosis and management of venous thromboembolism, and embolism of air, amniotic fluid, fat, or foreign material are discussed separately. (See "Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Epidemiology, etiology, and pathogenesis" and "Overview of acute pulmonary embolism in adults" and "Air embolism" and "Amniotic fluid embolism syndrome" and "Fat embolism syndrome" and "Foreign body granulomatosis".)
The terminology for both disorders is often used interchangeably by clinicians. However, pulmonary tumor embolism refers to the identification of tumor within pulmonary arteries and/or capillaries on pathologic lung samples, whereas pulmonary lymphangitic carcinomatosis refers to the identification of tumor in pulmonary lymphatic vessels. Since both entities are similarly evaluated and treated, distinguishing one from other has little clinical significance. (See "Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Epidemiology, etiology, and pathogenesis", section on 'Terminology'.)
While any malignancy can cause both disorders, the risk appears to be the greatest in patients with renal cell and hepatocellular carcinoma as well as adenocarcinoma of the breast, stomach, colon, and lung (table 1). The etiology and pathogenesis of these disorders are discussed separately. (See "Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Epidemiology, etiology, and pathogenesis", section on 'Etiology' and "Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Epidemiology, etiology, and pathogenesis", section on 'Pathogenesis'.)
The clinical manifestations of pulmonary tumor emboli and lymphangitic carcinomatosis are nonspecific with the majority of patients presenting with subacute, progressive, unexplained dyspnea [1-7]. Consequently, the diagnosis often remains unsuspected and not made until autopsy. Traditionally, it is thought that pulmonary tumor embolism is more likely to present with features suggestive of pulmonary hypertension or venous thromboembolism (dyspnea, hypoxemia, right heart strain, and clear lungs) while pulmonary lymphangitic carcinomatosis is more likely to present with the features of interstitial lung disease (eg, fever, bilateral infiltrates on chest imaging). However, clinical and pathological overlap between the entities is common. (See 'Definitive pathologic diagnosis' below.)
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- CLINICAL PRESENTATION
- Symptoms and signs
- Arterial blood gas
- Pulmonary function tests
- Electrocardiography and echocardiography
- DIAGNOSTIC APPROACH
- When to suspect tumor emboli or lymphangitic carcinomatosis
- Initial diagnostic tests
- Pursuing a histologic diagnosis
- - Right heart catheterization
- Pulmonary hypertension
- Pulmonary artery catheter aspiration
- - Bronchoscopy
- Bronchoalveolar lavage fluid
- Transbronchial biopsy
- - Surgical lung biopsy
- - Other
- Choosing among the tests
- DIFFERENTIAL DIAGNOSIS
- Venous thromboembolism
- Pulmonary hypertension
- Interstitial lung disease
- Definitive pathologic diagnosis
- - Pulmonary tumor embolism
- - Lymphangitic carcinomatosis
- Presumptive diagnosis
- SUMMARY AND RECOMMENDATIONS