Pulmonary tumor embolism and lymphangitic carcinomatosis in adults: Epidemiology, etiology, and pathogenesis
- Gerald L Weinhouse, MD
Gerald L Weinhouse, MD
- Assistant Professor of Medicine
- Harvard Medical School
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".)
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 . 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'.)
- Soares FA, Pinto AP, Landell GA, de Oliveira JA. Pulmonary tumor embolism to arterial vessels and carcinomatous lymphangitis. A comparative clinicopathological study. Arch Pathol Lab Med 1993; 117:827.
- Sakuma M, Fukui S, Nakamura M, et al. Cancer and pulmonary embolism: thrombotic embolism, tumor embolism, and tumor invasion into a large vein. Circ J 2006; 70:744.
- Roberts KE, Hamele-Bena D, Saqi A, et al. Pulmonary tumor embolism: a review of the literature. Am J Med 2003; 115:228.
- King MB, Harmon KR. Unusual forms of pulmonary embolism. Clin Chest Med 1994; 15:561.
- Chan CK, Hutcheon MA, Hyland RH, et al. Pulmonary tumor embolism: a critical review of clinical, imaging, and hemodynamic features. J Thorac Imaging 1987; 2:4.
- Peixoto LS, Valiante PM, Rodrigues RS, et al. An unusual cause of tree-in-bud pattern: pulmonary intravascular tumor embolism caused by chondrosarcoma. Lung 2015; 193:151.
- Hibino M, Akazawa K, Hikino K, Oe M. Pulmonary tumor embolism secondary to uterine corpus carcinosarcoma mimicking pulmonary thromboembolism. Intern Med 2012; 51:2603.
- Pérez Baztarrica G, Nieva N, Gariglio L, et al. Images in cardiovascular medicine. Primary cardiac lymphoma: a rare case of pulmonary tumor embolism. Circulation 2010; 121:2249.
- Liau CT, Jung SM, Lim KE, Shih HN. Pulmonary lymphangitic sarcomatosis from cutaneous angiosarcoma: an unusual presentation of diffuse interstitial lung disease. Jpn J Clin Oncol 2000; 30:37.
- Babu S, B S, M G, Salih S. A rare presentation of pulmonary lymphangitic carcinomatosis in cancer of lip: case report. World J Surg Oncol 2011; 9:77.
- Park JH, Seo HS, Park SK, et al. Spontaneous systemic tumor embolism caused by tumor invasion of pulmonary vein in a patient with advanced lung cancer. J Cardiovasc Ultrasound 2010; 18:148.
- Lindqvist C, Lepäntalo M, Jungell P. Lymphangitis carcinomatosa of the lungs. An unusual complication of oral cancer. Br J Oral Maxillofac Surg 1988; 26:228.
- Bassiri AG, Haghighi B, Doyle RL, et al. Pulmonary tumor embolism. Am J Respir Crit Care Med 1997; 155:2089.
- Lianidou ES, Strati A, Markou A. Circulating tumor cells as promising novel biomarkers in solid cancers. Crit Rev Clin Lab Sci 2014; 51:160.
- CHANDLER GN, TELLING M. Lymphangitis carcinomatosa. Br Med J 1952; 2:639.
- Hendin AS, Deveney CW. Postmortem demonstration of abnormal deep pulmonary lymphatic pathways in lymphangitic carcinomatosis. Cancer 1974; 33:1558.
- Soares FA, Landell GA, de Oliveira JA. Pulmonary tumour embolism from squamous cell carcinoma of the oesophagus. Eur J Cancer 1991; 27:495.
- Goldhaber SZ, Dricker E, Buring JE, et al. Clinical suspicion of autopsy-proven thrombotic and tumor pulmonary embolism in cancer patients. Am Heart J 1987; 114:1432.
- Shields, DJ, Edwards, WD. Pulmonary hypertension attributable to neoplastic emboli: An autopsy study of 20 cases and a review of the literature. Cardiovasc Pathol 1992; 1:279.
- Schmitt M, Harbeck N, Thomssen C, et al. Clinical impact of the plasminogen activation system in tumor invasion and metastasis: prognostic relevance and target for therapy. Thromb Haemost 1997; 78:285.
- Bruce DM, Heys SD, Eremin O. Lymphangitis carcinomatosa: a literature review. J R Coll Surg Edinb 1996; 41:7.