Abstract
The rate of malignant cardiac tumors is often wrongly reported as up to 30 %, when based on the data derived from pathology tertiary centers, where the most difficult cases are sent for expert opinion. Instead, primary malignant cardiac tumors are very rare representing nearly 10 % of all primary cardiac neoplasms. Sarcomas represent 95 % of these tumors, angiosarcoma, leiomyosarcoma, and unclassified sarcoma being the most common, followed by primary lymphoma and mesothelioma. All varieties of soft tissue sarcomas have been reported to arise from cardiac tissue.
There is no TNM classification for primary malignant cardiac tumors, and, due to their low frequency, there is no specific grading scheme for sarcomas. Thus, the general criteria proposed for grading malignant tumors of the soft tissues are utilized also for the cardiac one. According to the grading system for soft tissue sarcomas. a histological score from 1 to 3 is assigned to each of three parameters, i.e., tumor differentiation, mitotic rate, and amount of necrosis in untreated primary soft tissue sarcomas. The final grade is obtained by adding the three scores.
Primary cardiac sarcomas can occur at any age but are more frequently diagnosed between the third and fifth decades, and equally in men and women, whereas they are extremely rare in pediatric age. Surgical excision is the most effective treatment for primary cardiac malignancies. However, prognosis is very poor in spite of additional treatments, such as radiotherapy and chemotherapy, with a median survival less than 1 year and 80 % of patients already presenting diffuse infiltration of the heart and metastases at the time of diagnosis. A less-aggressive course seems related to the left atrium location, a low histologic grading with scarce cellular pleomorphism and low-mitotic activity, absence of necrosis, and absence of metastasis at diagnosis.
Keywords
- Synovial Sarcoma
- Malignant Mesothelioma
- Malignant Peripheral Nerve Sheath Tumor
- Malignant Fibrous Histiocytoma
- Solitary Fibrous Tumor
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References
Trawis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. World Health Organization classification of tumors: pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2004. p. 250.
Burke AP, Virmani R. Tumors of the heart and great vessels. 3rd ed. Washington, DC: Armed Forces Institute of Pathology; 1996.
Ramnarine IR, Davidson L, van Doorn CA. Primary cardiac carcinosarcoma: a rare, aggressive tumor. Ann Thorac Surg. 2001;72:927–9.
Fletcher CDM, Unni KK, Mertens F. World Health Organization classification of tumors: tumours of soft tissue and bone. Lyon: IARC Press; 2002.
Costa J. The grading and staging of soft tissue sarcomas. In: Fletcher CD, McKee PH, editors. Pathobiology of soft tissue tumors. Edinburg: Churchill Livingstone; 1990. p. 221–38.
Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, Goussot JF, David M, Bonichon F, Lagarde C. Soft tissue sarcomas of the adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer. 1984;33:37–42.
Coindre JM, Terrier P, Gouillou L, Le Doussal V, Collin F, Ranchere D, Sastre X, Vilain MO, Bonichon F, N’Guiyen Bui B. Predictive value of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group. Cancer. 2001;91:1914–26.
Uberfuhr P, Meiser B, Fuchs A, Schulze C, Reichenspurner H, Falk M, Weiss M, Wintersperger B, Issels R, Reichart B. Heart transplantation: an approach to treating primary cardiac sarcoma? J Heart Lung Transplant. 2002;21:1135–9.
Reardon MJ, Walkes JC, Benjamin R. Therapy insight: malignant primary cardiac tumors. Nat Rev Cardiol. 2006;3:548–53.
Butany J, Yu W. Cardiac angiosarcoma: two cases and a review of the literature. Can Cardiol. 2000;16:197–205.
Hamidi M, Moody JS, Weigel TL, Kozak KR. Primary cardiac sarcoma. Ann Thorac Surg. 2010;90:176–81.
Yu K, Liu Y, Wang H, Hu S, Long C. Epidemiological and pathological characteristics of cardiac tumors: a clinical study of 242 cases. Interact Cardiovasc Thorac Surg. 2007;6:636–9.
Simpson L, Kumar SK, Okuno SH, Schaff HV, Porrata LF, Buckner JC, Moynihan TJ. Malignant primary cardiac tumors: review of a single institution experience. Cancer. 2008;112:2440–6.
Mayer F, Aebert H, Rudert M, Königsrainer A, Horger M, Kanz L, Bamberg M, Ziemer G, Hartmann JT. Primary malignant sarcomas of the heart and great vessels in adult patients–a single-center experience. Oncologist. 2007;12:1134–42.
Glancy DL, Morales Jr JB, Roberts WC. Angiosarcoma of the heart. Am J Cardiol. 1968;21:413–9.
Rosenkranz ER, Murphy Jr DJ. Diagnosis and neonatal resection of right atrial angiosarcoma. Ann Thorac Surg. 1994;57:1014–5.
Adem C, Aubry MC, Tazelaar HD, Myers JL. Metastatic angiosarcoma masquerading as diffuse pulmonary hemorrhage: clinicopathologic analysis of 7 new patients. Arch Pathol Lab Med. 2001;125:1562–5.
Afzal MN, Alguacil-Garcia A. Primary cardiac angiosarcoma: clinical and pathological diagnostic problems. Can J Cardiol. 1997;13:293–6.
Poletti A, Cocco P, Valente M, Fasoli G, Chioin R, Thiene G. In vivo diagnosis of cardiac angiosarcoma by endomyocardial biopsy. Cardiovasc Pathol. 1993;2:89–91.
Araoz PA, Mulvagh SL, Tazelaar HD, Julsrud PR, Breen JF. CT and MR imaging of benign primary cardiac neoplasms with echocardiographic correlation. Radiographics. 2000;20:1303–19.
Economides EG, Singh A. Case of tumor neovascularization demonstrated by cardiac catheterization. Cathet Cardiovasc Diagn. 1998;43:451–3.
Garcia JM, Gonzalez R, Silva JM, Dominguez G, Vegazo IS, Gamallo C, Provencio M, Espana P, Bonilla F. Mutational status of K-ras and TP53 genes in primary sarcomas of the heart. Br J Cancer. 2000;82:1183–5.
Zu Y, Perle MA, Yan Z, Liu J, Kumar A, Waisman J. Chromosomal abnormalities and p53 gene mutation a cardiac angiosarcoma. Appl Immunohistochem Mol Morphol. 2001;9:24–8.
Kim MP, Correa AM, Blackmon S, Quiroga-Garza G, Weilbaecher D, Bruckner B, Ramlawi B, Rice DC, Vaporciyan AA, Reardon MJ. Outcomes after right-side heart sarcoma resection. Ann Thorac Surg. 2011;91:770–6.
Hermann MA, Shankerman RA, Edwards WD, Shub C, Schass HV. Primary cardiac angiosarcoma: a clinicopathologic study of six cases. J Thorac Cardiovasc Surg. 1992;103:655–64.
Burke AP, Tazelaar H, Butany JW, EI-Demellawy D, Loire R, Geva T, Bonilla F, Galvin JR, Veinot JP, Virmani R, Kamiya H, Watanabe G, Grandmougin D, Horimoto M, Hiraga H. Cardiac sarcomas. In: Trawis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. World Health Organization classification of tumors: pathology and genetics of tumours of the lung pleura thymus and heart. Lyon: IARC Press; 2004. p. 273–81.
Van Bernal JF, Garcia Alberdi E, Gutierrez JA, Garijo MF. Incidental in vivo detection of an epitheliod hemangioendothelioma of the mitral valve. Pathol Int. 2005;55:644–8.
Boudousquie AC, Lawce HJ, Sherman R, Olson S, Magenis RE, Corless CL. Complex translocation (7; 22) identified in an epithelioid hemangioendothelioma. Cancer Genetic Cytogenet. 1996;92:116–21.
Mendlick MR, Nelson M, Pickering D, Johansson SL, Seemayer TA, Neff JR, Vergara G, Rosenthal H, Bridge JA. Translocation t(1; 3)(p36.3; q25) is a nonrandom aberration in epithelioid hemangioendothelioma. Am J Surg Pathol. 2001;25:684–7.
Marchiano D, Fisher F, Hofstetter S. Epithelioid hemangioendothelioma of the heart with distant metastases: A case report and literature review. J Cardiovasc Surg. 1993;34:529–33.
Whorton CM. Primary malignant tumors of the heart. Cancer. 1949;2:245–60.
Bear PA, Moodie DS. Malignant primary cardiac tumors. The Cleveland Clinic experience, 1956 to 1986. Chest. 1987;92:860–2.
Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of Minnesota. Thorac Cardiovasc Surg. 1990;38:183–91.
Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardiothorac Surg. 1997;12:730–7.
Okamoto K, Kato S, Katsuki S, Wada Y, Toyozumi Y, Morimatsu M, Aoyagi S, Imaizumi T. Malignant fibrous histiocytoma of the heart: case report and review of 46 cases in the literature. Intern Med. 2001;40:1222–6.
Laya MB, Mailliard JA, Bewtra C, Levin HS. Malignant fibrous histiocytoma of the heart. A case report and review of the literature. Cancer. 1987;59:1026–31.
Terashima K, Aoyama K, Nihei K, Nito T, Imai Y, Takahashi K, Daidoji S. Malignant fibrous histiocytoma of the heart. Cancer. 1983;52:1919–26.
Mairal A, Terrier P, Chibon F, Sastre X, Lecesne A, Aurias A. Loss of chromosome 13 is the most frequent genomic imbalance in malignant fibrous histiocytoma. A comparative genomic hybridization analysis of a series of 30 cases. Cancer Genet Cytogenet. 1999;111:134–8.
Chibon F, Mairal A, Freneaux P, Terrier P, Coindre JM, Sastre X, Aurias A. The RB1 gene is the target of chromosome 13 deletions in malignant fibrous histiocytoma. Cancer Res. 2000;60:6339–45.
Simons A, Schepens M, Jeuken J, Sprenger S, van de Zande G, Bjerkehagen B, Forus A, Weibolt V, Molenaar I, van den Berg E, Myklebost O, Bridge J, van Kessel AG, Suijkerbuijk R. Frequent loss of 9p21 (P16(INK4A)) and other genomic imbalances in human malignant fibrous histiocytoma. Cancer Genet Cytogenet. 2000;118:89–98.
Gowdamarajan A, Michler RE. Therapy for primary cardiac tumors: is there a role for heart transplantation? Curr Opin Cardiol. 2000;15:121–5.
Talbot SN, Taub RN, Keohan ML, Edwards N, Galantowicz ME, Schulman LL. Combined heart and lung transplantation for unresectable primary cardiac sarcoma. J Thorac Cardiovasc Surg. 2002;124:1145–8.
Reardon MJ, De Felice CA, Sheinbaum R, Baldwin JC. Cardiac autotransplant for surgical treatment of a malignant neoplasm. Ann Thorac Surg. 1999;67:1793–5.
Reynard JS, Gregoratos G, Gordon MJ, Bloor CM. Primary osteosarcoma of the heart. Am Heart J. 1985;109:598–600.
Burke A, Virmani R. Osteosarcomas of the heart. Am J Surg Pathol. 1991;15:289–95.
Yamagishi M, Bando K, Furuichi S, Ishibashi-Ueda H, Yutani C, Miyatake K. Images in cardiovascular medicine: primary cardiac osteosarcoma in right ventricular outflow tract. Circulation. 2000;101:2220–1.
Basso C, Stefani A, Calabrese F, Fasoli G, Valente M. Primary right atrial fibrosarcoma diagnosed by endocardial biopsy. Am Heart J. 1996;131:399–402.
Okada M, Ohta T, Yasuoka S, Matsuda S, Shida T, Nakamura K, Asada S. Surgical management of intracavitary tumors. A review of fifteen patients and current status in Japan. J Cardiovasc Surg. 1986;27:641–9.
Hui KS, Green LK, Schmidt WA. Primary cardiac rhabdomyosarcoma: definition of a rare entity. Am J Cardiovasc Pathol. 1988;2:19–29.
Donsbeck AV, Ranchere D, Coindre JM, Le Gall F, Cordier JF, Loire R. Primary cardiac sarcomas: an immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology. 1999;34:295–304.
Tazelaar HD, Locke TJ, McGregor CG. Pathology of surgically excised primary cardiac tumors. Mayo Clin Proc. 1992;67:957–65.
Thiene G, Miraglia G, Menghetti L, Nava A, Rossi L. Multiple lesions of the conduction system in a case of cardiac rhabdomyosarcoma with complex arrhythmias. An anatomic and clinical study. Chest. 1976;70:378–81.
Hajar R, Roberts WC, Folger Jr GM. Embryonal botryoid rhabdomyosarcoma of the mitral valve. Am J Cardiol. 1986;57:376.
Scott RS, Jagirdar J. Right atrial botryoid rhabdomyosarcoma in an adult patient with recurrent pleomorphic rhabdomyosarcomas following doxorubicin therapy. Ann Diagn Pathol. 2007;11:274–6.
Orsmond GS, Knight L, Dehner LP, Nicoloff DM, Nesbitt M, Bessinger Jr FB. Alveolar rhabdomyosarcoma involving the heart. An echocardiographic, angiographic and pathologic study. Circulation. 1976;54:837–43.
Quintanilla-Martinez L, Wilkins Jr EW, Choi N, Efird J, Hug E, Harris NL. Thymoma. Histologic subclassication is an independent prognostic factor. Cancer. 1994;74:606–17.
Garcia JM, Gonzalez R, Silva JM, Dominguez G, Vegazo IS, Gamallo C, Provencio M, Espana P, Bonilla F. Mutational status of K-ras and TP53 genes in primary sarcomas of the heart. Br J Cancer. 2000;82:1183–5.
Grandmougin D, Fayad G, Decoene C, Pol A, Warembourg H. Total orthotopic heart transplantation for primary cardiac rhabdomyosarcoma: factors influencing long-term survival. Ann Thorac Surg. 2001;71:1438–41.
Mazzola A, Spano JP, Valente M, Gregoriani R, Villani C, Di Eusanio M, Ciocca M, Minuti U, Giancola R, Basso C, Thiene G. Leiomyosarcoma of the left atrium mimicking a left atrial myxoma. J Thorac Cardiovasc Surg. 2006;131:224–6.
Pessotto R, Silvestre G, Luciani GB, Anselmi M, Pasini F, Santini F, Mazzucco A. Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy. Int J Cardiol. 1997;60:91–4.
Hazelbag HM, Szuhai K, Tanke HJ, Rosenberg C, Hogendoorn PC. Primary synovial sarcoma of the heart: a cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH of a case with a complex karyotype. Mod Pathol. 2004;17:1434–9.
Guillou L, Coindre JM, Bonichon F, Nguyen BB, Terrier P, Collin F, Vilain MO, Mandard AM, le Doussal V, Benhattar J. Detection of the synovial sarcoma translocation t(X;18) (SYT; SSX) in paraffin-embedded tissues using reverse transcriptase-polymerase chain reaction. A reliable and powerful diagnostic tool for pathologists. A molecular analysis of 221 mesenchymal tumors fixed in different fixatives. Hum Pathol. 2001;32:105–12.
Amary MF, Berisha F, Bernardi Fdel C, Herbert A, James M, Reis-Filho JS, Fisher C, Nicholson AG, Tirabosco R, Diss TC, Flanagan AM. Detection of SS18-SSX fusion transcripts in formalin-fixed paraffin-embedded neoplasms: analysis of conventional RT-PCR, qRT-PCR and dual color FISH as diagnostic tools for synovial sarcoma. Mod Pathol. 2007;20:482–96.
Paraf F, Bruneval P, Balaton A, Deloche A, Mikol J, Maitre F, Scholl JM, De Saint-Maur PP, Camilleri JP. Primary liposarcoma of the heart. Am J Cardiovac Pathol. 1990;3:175–80.
Nzayinambaho K, Noel H, Cosyns J, Sonnet J, Chalant C. Primary cardiac liposarcoma simulating a left atrial myxoma. Thorac Cardiovasc Surg. 1985;33:193–5.
Can C, Arpaci F, Celasum B, Gunham O, Finci R. Primary pericardial liposarcoma presenting with cardiac tamponade and multiple organ metastates (Letter). Chest. 1993;103:328.
Cairns P, Butany J, Fulop J, Ralowski H, Hassaram S. Cardiac presentation of non-Hodgkin’s lymphoma. Arch Pathol Lab Med. 1987;111:80–3.
Petrich A, Cho SI, Billett H. Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns. Cancer. 2011;117:581–9.
Rolla G, Bertero MT, Pastena G, Tartaglia N, Corradi F, Casabona R, Motta M, Caligaris-cappio F. Primary lymphoma of the heart. A case report and review of the literature. Leuk Res. 2002;26:117–20.
Bortolotti U, Calabro F, Loy M, Fasoli G, Altavilla G, Marchese D. Giant intrapericardial solitary fibrous tumor. Ann Thorac Surg. 1992;54:1219–20.
Corgnati G, Drago S, Bonamini R, Trevi GP, Carra R, Di Summa M. Solitary fibrous tumor of the pericardium presenting itself as a pericardial effusion and right ventricular obstruction. J Cardiovasc Surg. 2004;45:393–4.
Hanau CA, Miettinen M. Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol. 1995;26:440–9.
Krismann M, Muller KM. Malignant mesothelioma of the pleura, pericardium and peritoneum. 1: Etiology, pathogenesis, pathology. Chirurg. 2000;71:877–86.
Churg A, Warnock ML, Bensch KG. Malignant mesothelioma arising after direct application of asbestos and fiber glass to the pericardium. Am Rev Respir Dis. 1978;118:419–24.
Small GR, Nicolson M, Buchan K. Pericardial malignant mesothelioma: a latent complication of radiotherapy? Eur J Cardiothor Sur. 2008;33:745–7.
Loire R, Tabib A. Malignant mesothelioma of the pericardium. An anatomo-clinical study of 10 cases. Arch Mal Coeur Vaiss. 1994;87:255–62.
McKenney H. Congenital teratomas. Am J Surg Pathol. 2005;29:29–38.
Carachi R, Campbell PE, Chow CW, Mee BB. Alpha-feto-protein-(AFP-)-secreting intra-pericardial teratoma–report of a case diagnosed on CT scanning. Z Kinderchir. 1986;41:369–70.
Liang TC, Lu MY, Chen SJ, Lu FL, Lin KH. Cardiac tamponade caused by intrapericardial yolk sac tumor in a boy. J Formos Med Assoc. 2002;101:355–8.
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Valente, M., Rizzo, S., Leone, O., Basso, C. (2013). Primary Malignant Tumors of the Heart. In: Basso, C., Valente, M., Thiene, G. (eds) Cardiac Tumor Pathology. Current Clinical Pathology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-143-1_6
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