Pericardial disease associated with malignancy
- Barry A Borlaug, MD
Barry A Borlaug, MD
- Associate Professor of Medicine
- Mayo Clinic College of Medicine
- Malcolm M DeCamp, MD
Malcolm M DeCamp, MD
- Fowler McCormick Professor of Surgery
- Northwestern University Feinberg School of Medicine
- Section Editor
- Martin M LeWinter, MD
Martin M LeWinter, MD
- Section Editor — Myopericardial Disease
- Professor of Medicine and Molecular Physiology and Biophysics
- University of Vermont
- Deputy Editors
- Brian C Downey, MD, FACC
Brian C Downey, MD, FACC
- Deputy Editor — Cardiology
- Assistant Professor of Medicine
- Tufts University School of Medicine
- Diane MF Savarese, MD
Diane MF Savarese, MD
- Senior Deputy Editor — UpToDate
- Deputy Editor — Oncology and Palliative Care
- Clinical Instructor of Medicine
- Harvard Medical School
Malignant involvement of the pericardium is detected in 1 to 20 percent of cancer cases in autopsy studies [1,2]. Direct involvement of the myocardium is much less frequent, either by metastatic or primary tumors [3,4]. In two large autopsy series [2,5], the incidence of any cardiac involvement was 11 and 12 percent, respectively. Of these, 76 percent had pericardial involvement, and 34 percent had an effusion .
The most common metastatic tumor involving the pericardium is lung cancer; others include breast and esophageal cancer, melanoma, lymphoma, and leukemia. Although AIDS-related Kaposi's sarcoma (KS) has been an important cause of neoplastic pericardial disease, the incidence of KS has declined dramatically since the advent of potent antiretroviral therapy. (See 'Etiology and pathogenesis' below and "AIDS-related Kaposi sarcoma: Clinical manifestations and diagnosis", section on 'Epidemiology and risk factors' and "Cardiac and vascular disease in HIV-infected patients", section on 'Pericardial disease'.)
Here we will discuss the presentation and management of pericardial disease associated with malignancy. An overview of the etiology of pericardial disease, and the clinical presentation, diagnosis and management of constrictive/restrictive pericardial disease related to cancer or its treatment is presented elsewhere. (See "Etiology of pericardial disease" and "Constrictive pericarditis".)
Malignant involvement of the pericardium can be manifested as pericarditis, pericardial effusion, cardiac tamponade, or pericardial constriction (constrictive pericarditis).
Pericarditis — Pericarditis is an acute inflammatory process characterized clinically by chest pain, pericardial friction rub, and widespread saddle-shaped or concave up ST segment elevation on the electrocardiogram (ECG) (waveform 1). At least two of these features, with or without an accompanying pericardial effusion, should be present to make the diagnosis. The chest pain is characteristically pleuritic, radiates to the trapezius ridge, and is worse in the supine position. These factors help to differentiate it from pain due to myocardial ischemia. (See "Clinical presentation and diagnostic evaluation of acute pericarditis".)
- Maisch B, Ristic A, Pankuweit S. Evaluation and management of pericardial effusion in patients with neoplastic disease. Prog Cardiovasc Dis 2010; 53:157.
- Klatt EC, Heitz DR. Cardiac metastases. Cancer 1990; 65:1456.
- Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med 1993; 117:1027.
- MacGee W. Metastatic and invasive tumours involving the heart in a geriatric population: a necropsy study. Virchows Arch A Pathol Anat Histopathol 1991; 419:183.
- Abraham KP, Reddy V, Gattuso P. Neoplasms metastatic to the heart: review of 3314 consecutive autopsies. Am J Cardiovasc Pathol 1990; 3:195.
- Ben-Horin S, Bank I, Guetta V, Livneh A. Large symptomatic pericardial effusion as the presentation of unrecognized cancer: a study in 173 consecutive patients undergoing pericardiocentesis. Medicine (Baltimore) 2006; 85:49.
- Atar S, Chiu J, Forrester JS, Siegel RJ. Bloody pericardial effusion in patients with cardiac tamponade: is the cause cancerous, tuberculous, or iatrogenic in the 1990s? Chest 1999; 116:1564.
- Mann T, Brodie BR, Grossman W, McLaurin L. Effusive-constrictive hemodynamic pattern due to neoplastic involvement of the pericardium. Am J Cardiol 1978; 41:781.
- Wilkes JD, Fidias P, Vaickus L, Perez RP. Malignancy-related pericardial effusion. 127 cases from the Roswell Park Cancer Institute. Cancer 1995; 76:1377.
- Gornik HL, Gerhard-Herman M, Beckman JA. Abnormal cytology predicts poor prognosis in cancer patients with pericardial effusion. J Clin Oncol 2005; 23:5211.
- Sampat K, Rossi A, Garcia-Gutierrez V, et al. Characteristics of pericardial effusions in patients with leukemia. Cancer 2010; 116:2366.
- Barbaric D, Holley D, Lau KC, McCowage G. It is ALL in the heart: a patient with acute lymphoblastic leukemia and cardiac infiltration at time of diagnosis. Leuk Lymphoma 2002; 43:2417.
- Savoia P, Fierro MT, Zaccagna A, Bernengo MG. Metastatic melanoma of the heart. J Surg Oncol 2000; 75:203.
- Chen Y, Brennessel D, Walters J, et al. Human immunodeficiency virus-associated pericardial effusion: report of 40 cases and review of the literature. Am Heart J 1999; 137:516.
- Gouny P, Lancelin C, Girard PM, et al. Pericardial effusion and AIDS: benefits of surgical drainage. Eur J Cardiothorac Surg 1998; 13:165.
- Porte HL, Janecki-Delebecq TJ, Finzi L, et al. Pericardoscopy for primary management of pericardial effusion in cancer patients. Eur J Cardiothorac Surg 1999; 16:287.
- Posner MR, Cohen GI, Skarin AT. Pericardial disease in patients with cancer. The differentiation of malignant from idiopathic and radiation-induced pericarditis. Am J Med 1981; 71:407.
- Buck M, Ingle JN, Giuliani ER, et al. Pericardial effusion in women with breast cancer. Cancer 1987; 60:263.
- Edoute Y, Kuten A, Ben-Haim SA, et al. Symptomatic pericardial effusion in breast cancer patients: the role of fluid cytology. J Surg Oncol 1990; 45:265.
- Imazio M, Demichelis B, Parrini I, et al. Relation of acute pericardial disease to malignancy. Am J Cardiol 2005; 95:1393.
- Permanyer-Miralda G, Sagristá-Sauleda J, Soler-Soler J. Primary acute pericardial disease: a prospective series of 231 consecutive patients. Am J Cardiol 1985; 56:623.
- Zayas R, Anguita M, Torres F, et al. Incidence of specific etiology and role of methods for specific etiologic diagnosis of primary acute pericarditis. Am J Cardiol 1995; 75:378.
- Imazio M, Cecchi E, Demichelis B, et al. Indicators of poor prognosis of acute pericarditis. Circulation 2007; 115:2739.
- Cameron J, Oesterle SN, Baldwin JC, Hancock EW. The etiologic spectrum of constrictive pericarditis. Am Heart J 1987; 113:354.
- Ling LH, Oh JK, Schaff HV, et al. Constrictive pericarditis in the modern era: evolving clinical spectrum and impact on outcome after pericardiectomy. Circulation 1999; 100:1380.
- Bertog SC, Thambidorai SK, Parakh K, et al. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J Am Coll Cardiol 2004; 43:1445.
- Corey GR, Campbell PT, Van Trigt P, et al. Etiology of large pericardial effusions. Am J Med 1993; 95:209.
- Sagristà-Sauleda J, Mercé J, Permanyer-Miralda G, Soler-Soler J. Clinical clues to the causes of large pericardial effusions. Am J Med 2000; 109:95.
- Gross JL, Younes RN, Deheinzelin D, et al. Surgical management of symptomatic pericardial effusion in patients with solid malignancies. Ann Surg Oncol 2006; 13:1732.
- Wang ZJ, Reddy GP, Gotway MB, et al. CT and MR imaging of pericardial disease. Radiographics 2003; 23 Spec No:S167.
- Bellon RJ, Wright WH, Unger EC. CT-guided pericardial drainage catheter placement with subsequent pericardial sclerosis. J Comput Assist Tomogr 1995; 19:672.
- Wiener HG, Kristensen IB, Haubek A, et al. The diagnostic value of pericardial cytology. An analysis of 95 cases. Acta Cytol 1991; 35:149.
- Meyers DG, Meyers RE, Prendergast TW. The usefulness of diagnostic tests on pericardial fluid. Chest 1997; 111:1213.
- Zoppi JA, Pellicer EM, Sundblad AS. Diagnostic value of p53 protein in the study of serous effusions. Acta Cytol 1995; 39:721.
- Gong Y, Sun X, Michael CW, et al. Immunocytochemistry of serous effusion specimens: a comparison of ThinPrep vs cell block. Diagn Cytopathol 2003; 28:1.
- Mayall F, Heryet A, Manga D, Kriegeskotten A. p53 immunostaining is a highly specific and moderately sensitive marker of malignancy in serous fluid cytology. Cytopathology 1997; 8:9.
- Bardales RH, Stanley MW, Schaefer RF, et al. Secondary pericardial malignancies: a critical appraisal of the role of cytology, pericardial biopsy, and DNA ploidy analysis. Am J Clin Pathol 1996; 106:29.
- Maisch B, Pankuweit S, Brilla C, et al. Intrapericardial treatment of inflammatory and neoplastic pericarditis guided by pericardioscopy and epicardial biopsy--results from a pilot study. Clin Cardiol 1999; 22:I17.
- Nugue O, Millaire A, Porte H, et al. Pericardioscopy in the etiologic diagnosis of pericardial effusion in 141 consecutive patients. Circulation 1996; 94:1635.
- Iqbal J, Liu T, Mapow B, et al. Importance of flow cytometric analysis of serous effusions in the diagnosis of hematopoietic neoplasms in patients with prior hematopoietic malignancies. Anal Quant Cytol Histol 2010; 32:161.
- Czader M, Ali SZ. Flow cytometry as an adjunct to cytomorphologic analysis of serous effusions. Diagn Cytopathol 2003; 29:74.
- Hurrell DG, Nishimura RA, Higano ST, et al. Value of dynamic respiratory changes in left and right ventricular pressures for the diagnosis of constrictive pericarditis. Circulation 1996; 93:2007.
- Laham RJ, Cohen DJ, Kuntz RE, et al. Pericardial effusion in patients with cancer: outcome with contemporary management strategies. Heart 1996; 75:67.
- Spodick DH. Acute cardiac tamponade. N Engl J Med 2003; 349:684.
- Vaitkus PT, Herrmann HC, LeWinter MM. Treatment of malignant pericardial effusion. JAMA 1994; 272:59.
- Tsang TS, Seward JB, Barnes ME, et al. Outcomes of primary and secondary treatment of pericardial effusion in patients with malignancy. Mayo Clin Proc 2000; 75:248.
- Virk SA, Chandrakumar D, Villanueva C, et al. Systematic review of percutaneous interventions for malignant pericardial effusion. Heart 2015; 101:1619.
- El Haddad D, Iliescu C, Yusuf SW, et al. Outcomes of Cancer Patients Undergoing Percutaneous Pericardiocentesis for Pericardial Effusion. J Am Coll Cardiol 2015; 66:1119.
- McDonald JM, Meyers BF, Guthrie TJ, et al. Comparison of open subxiphoid pericardial drainage with percutaneous catheter drainage for symptomatic pericardial effusion. Ann Thorac Surg 2003; 76:811.
- Allen KB, Faber LP, Warren WH, Shaar CJ. Pericardial effusion: subxiphoid pericardiostomy versus percutaneous catheter drainage. Ann Thorac Surg 1999; 67:437.
- Kopecky SL, Callahan JA, Tajik AJ, Seward JB. Percutaneous pericardial catheter drainage: report of 42 consecutive cases. Am J Cardiol 1986; 58:633.
- Patel N, Rafique AM, Eshaghian S, et al. Retrospective comparison of outcomes, diagnostic value, and complications of percutaneous prolonged drainage versus surgical pericardiotomy of pericardial effusion associated with malignancy. Am J Cardiol 2013; 112:1235.
- Celermajer DS, Boyer MJ, Bailey BP, Tattersall MH. Pericardiocentesis for symptomatic malignant pericardial effusion: a study of 36 patients. Med J Aust 1991; 154:19.
- Liu G, Crump M, Goss PE, et al. Prospective comparison of the sclerosing agents doxycycline and bleomycin for the primary management of malignant pericardial effusion and cardiac tamponade. J Clin Oncol 1996; 14:3141.
- Maher EA, Shepherd FA, Todd TJ. Pericardial sclerosis as the primary management of malignant pericardial effusion and cardiac tamponade. J Thorac Cardiovasc Surg 1996; 112:637.
- Davis S, Rambotti P, Grignani F. Intrapericardial tetracycline sclerosis in the treatment of malignant pericardial effusion: an analysis of thirty-three cases. J Clin Oncol 1984; 2:631.
- Lashevsky I, Ben Yosef R, Rinkevich D, et al. Intrapericardial minocycline sclerosis for malignant pericardial effusion. Chest 1996; 109:1452.
- Patz EF Jr, McAdams HP, Erasmus JJ, et al. Sclerotherapy for malignant pleural effusions: a prospective randomized trial of bleomycin vs doxycycline with small-bore catheter drainage. Chest 1998; 113:1305.
- Maruyama R, Yokoyama H, Seto T, et al. Catheter drainage followed by the instillation of bleomycin to manage malignant pericardial effusion in non-small cell lung cancer: a multi-institutional phase II trial. J Thorac Oncol 2007; 2:65.
- Kunitoh H, Tamura T, Shibata T, et al. A randomised trial of intrapericardial bleomycin for malignant pericardial effusion with lung cancer (JCOG9811). Br J Cancer 2009; 100:464.
- van Belle SJ, Volckaert A, Taeymans Y, et al. Treatment of malignant pericardial tamponade with sclerosis induced by instillation of bleomycin. Int J Cardiol 1987; 16:155.
- Buchanan CL, Sullivan VV, Lampman R, Kulkarni MG. Pericardiocentesis with extended catheter drainage: an effective therapy. Ann Thorac Surg 2003; 76:817.
- Lin MT, Yang PC, Luh KT. Constrictive pericarditis after sclerosing therapy with mitomycin C for malignant pericardial effusion: report of a case. J Formos Med Assoc 1994; 93:250.
- Moores DW, Allen KB, Faber LP, et al. Subxiphoid pericardial drainage for pericardial tamponade. J Thorac Cardiovasc Surg 1995; 109:546.
- Cullinane CA, Paz IB, Smith D, et al. Prognostic factors in the surgical management of pericardial effusion in the patient with concurrent malignancy. Chest 2004; 125:1328.
- Park JS, Rentschler R, Wilbur D. Surgical management of pericardial effusion in patients with malignancies. Comparison of subxiphoid window versus pericardiectomy. Cancer 1991; 67:76.
- Olson JE, Ryan MB, Blumenstock DA. Eleven years' experience with pericardial-peritoneal window in the management of malignant and benign pericardial effusions. Ann Surg Oncol 1995; 2:165.
- Hankins JR, Satterfield JR, Aisner J, et al. Pericardial window for malignant pericardial effusion. Ann Thorac Surg 1980; 30:465.
- DeCamp MM Jr, Mentzer SJ, Swanson SJ, Sugarbaker DJ. Malignant effusive disease of the pleura and pericardium. Chest 1997; 112:291S.
- Wang HJ, Hsu KL, Chiang FT, et al. Technical and prognostic outcomes of double-balloon pericardiotomy for large malignancy-related pericardial effusions. Chest 2002; 122:893.
- Galli M, Politi A, Pedretti F, et al. Percutaneous balloon pericardiotomy for malignant pericardial tamponade. Chest 1995; 108:1499.
- Jalisi FM, Morise AP, Haque R, Jain AC. Primary percutaneous balloon pericardiotomy. W V Med J 2004; 100:102.
- Ziskind AA, Pearce AC, Lemmon CC, et al. Percutaneous balloon pericardiotomy for the treatment of cardiac tamponade and large pericardial effusions: description of technique and report of the first 50 cases. J Am Coll Cardiol 1993; 21:1.
- Swanson N, Mirza I, Wijesinghe N, Devlin G. Primary percutaneous balloon pericardiotomy for malignant pericardial effusion. Catheter Cardiovasc Interv 2008; 71:504.
- DeValeria PA, Baumgartner WA, Casale AS, et al. Current indications, risks, and outcome after pericardiectomy. Ann Thorac Surg 1991; 52:219.
- Bishiniotis TS, Antoniadou S, Katseas G, et al. Malignant cardiac tamponade in women with breast cancer treated by pericardiocentesis and intrapericardial administration of triethylenethiophosphoramide (thiotepa). Am J Cardiol 2000; 86:362.
- Colleoni M, Martinelli G, Beretta F, et al. Intracavitary chemotherapy with thiotepa in malignant pericardial effusions: an active and well-tolerated regimen. J Clin Oncol 1998; 16:2371.
- Martinoni A, Cipolla CM, Cardinale D, et al. Long-term results of intrapericardial chemotherapeutic treatment of malignant pericardial effusions with thiotepa. Chest 2004; 126:1412.
- Moriya T, Takiguchi Y, Tabeta H, et al. Controlling malignant pericardial effusion by intrapericardial carboplatin administration in patients with primary non-small-cell lung cancer. Br J Cancer 2000; 83:858.
- Tomkowski WZ, Wiśniewska J, Szturmowicz M, et al. Evaluation of intrapericardial cisplatin administration in cases with recurrent malignant pericardial effusion and cardiac tamponade. Support Care Cancer 2004; 12:53.
- Maisch B, Ristić AD, Pankuweit S, et al. Neoplastic pericardial effusion. Efficacy and safety of intrapericardial treatment with cisplatin. Eur Heart J 2002; 23:1625.
- Musch E, Gremmler B, Nitsch J, et al. Intrapericardial instillation of mitoxantrone in palliative therapy of malignant pericardial effusion. Onkologie 2003; 26:135.
- Norum J, Lunde P, Aasebø U, Himmelmann A. Mitoxantrone in malignant pericardial effusion. J Chemother 1998; 10:399.
- Kawashima O, Kurihara T, Kamiyoshihara M, et al. Management of malignant pericardial effusion resulting from recurrent cancer with local instillation of aclarubicin hydrochloride. Am J Clin Oncol 1999; 22:396.
- Dequanter D, Lothaire P, Berghmans T, Sculier JP. Severe pericardial effusion in patients with concurrent malignancy: a retrospective analysis of prognostic factors influencing survival. Ann Surg Oncol 2008; 15:3268.
- Dosios T, Theakos N, Angouras D, Asimacopoulos P. Risk factors affecting the survival of patients with pericardial effusion submitted to subxiphoid pericardiostomy. Chest 2003; 124:242.
- Piehler JM, Pluth JR, Schaff HV, et al. Surgical management of effusive pericardial disease. Influence of extent of pericardial resection on clinical course. J Thorac Cardiovasc Surg 1985; 90:506.
- Neragi-Miandoab S, Linden PA, Ducko CT, et al. VATS pericardiotomy for patients with known malignancy and pericardial effusion: survival and prognosis of positive cytology and metastatic involvement of the pericardium: a case control study. Int J Surg 2008; 6:110.
- Wang PC, Yang KY, Chao JY, et al. Prognostic role of pericardial fluid cytology in cardiac tamponade associated with non-small cell lung cancer. Chest 2000; 118:744.
- Girardi LN, Ginsberg RJ, Burt ME. Pericardiocentesis and intrapericardial sclerosis: effective therapy for malignant pericardial effusions. Ann Thorac Surg 1997; 64:1422.
- García-Riego A, Cuiñas C, Vilanova JJ. Malignant pericardial effusion. Acta Cytol 2001; 45:561.
- CLINICAL SYNDROMES
- Pericardial effusion
- - Cardiac tamponade
- Constrictive pericarditis
- ETIOLOGY AND PATHOGENESIS
- Pericardial disease as the first manifestation of cancer
- DIAGNOSTIC EVALUATION
- Imaging studies
- - Chest radiography
- - Echocardiography
- - CT and MRI
- Cytology, flow cytometry, and pericardial biopsy
- Cardiac catheterization
- Acute management of the effusion
- Prevention of effusion reaccumulation
- - Prolonged catheter drainage
- - Pericardial sclerosis
- - Surgical decompression of the pericardium
- - Balloon pericardiotomy
- - Management of pericardial constriction
- - Intrapericardial chemotherapy
- Prognosis and status of the underlying malignancy
- MISCELLANEOUS PERICARDIAL LESIONS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS