Clinical Classification of Cardiovascular Tumors and Tumor-Like Lesions, and Its Incidences

View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Springer - Publisher Connector Gen Thorac Cardiovasc Surg (2013) 61:435–447 DOI 10.1007/s11748-013-0214-8 CURRENT TOPICS REVIEW ARTICLE Clinical classification of cardiovascular tumors and tumor-like lesions, and its incidences Jun Amano • Jun Nakayama • Yasuo Yoshimura • Uichi Ikeda Received: 14 November 2012 / Published online: 5 March 2013 Ó The Author(s) 2013. This article is published with open access at Springerlink.com Abstract Tumors of the heart and the great vessels are originated from the cardiovascular system, and various very rare disease, and there are many disorders such as metastatic tumors from nearby organs, distant lesions, and tumors originated from the heart and great vessels, meta- intravascular extension tumors to the heart were reported. static tumors, and tumor-like lesions which do not fit into Based on the new classification, we reviewed epidemiology the usual concept of tumor or neoplasm; thus, it is very and incidence of cardiovascular tumors. Metastatic tumors difficult to classify these tumors. We proposed a new are more frequent than tumors originated from the heart classification of cardiovascular tumors for clinical use and great vessels, and cardiac myxoma is the most frequent based on the accumulated biological analyses and clinical tumors in all cardiac tumors. data of the reported literatures and our own study as benign tumors, malignant tumors, ectopic hyperplasia/ectopic Keywords Cardiac tumor Á Metastatic cardiac tumors Á tumors/others, and tumors of great vessels, with reference Classification Á Incidence Á Cardiac myxoma to the series of Atlas of tumor pathology of the Armed Forces Institute of Pathology and the recent World Health Organization classification of cardiac tumors issued in 2004. More than 50 disorders have been reported as tumors Introduction Tumors of the cardiovascular system, benign or malignant, The review was submitted at the invitation of the editorial committee. are very rare disease, and there are many disorders which do not fit into the concept of tumor or neoplasm; thus, it is J. Amano (&) very difficult to classify the tumors of the heart and great Department of Cardiovascular Surgery, vessels. As tumors of other organ, tumor of the heart and Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, Japan great vessels are classified as tumors originated from the e-mail: [email protected] heart and great vessels, or secondary/metastatic tumor with invasion nearby organs such as lung or metastatic tumors J. Nakayama that occur in the distant organ. In addition, tumors origi- Department of Molecular Pathology, Shinshu University Graduate School of Medicine, nated from the heart are classified by the site of tumor 3-1-1 Asahi, Matsumoto, Nagano, Japan location such as tumor of the heart, cardiac septum, pericardium, or great vessels, and classified by cell type con- Y. Yoshimura stituting the tumor such as hyperplasia, hamartoma, cyst, or Department of Orthopaedic Surgery, Shinshu University School of Medicine, benign or malignant, and also classified by histological 3-1-1 Asahi, Matsumoto, Nagano, Japan features such as mesenchymal, epithelial, and serous membrane (mesothelium). However, there is no established U. Ikeda standard method of classification of tumors of the heart and Department of Cardiovascular Medicine, Shinshu University School of Medicine, great vessels up to now. In this review, we propose a new 3-1-1 Asahi, Matsumoto, Nagano, Japan clinical classification for tumors of the cardiovascular 123 436 Gen Thorac Cardiovasc Surg (2013) 61:435–447 system, and summarize its epidemiology and incidence Table 1 Classification of ‘‘tumors of the heart and great vessels’’ by from the reported literatures and our own study. the Armed Forces Institute of Pathology Benign cardiac tumors Tumors of unknown histogenesis Classification of tumors of the heart and great vessels Myxoma Papillary fibroelastoma Tumors originated from the heart and great vessels are Tumors of cardiac muscle found at an incidence of about 0.02 % of autopsy. Among Rhabdomyoma these cardiovascular tumors, 70 % of these tumors and Histiocytoid cardiomyopathy (purkinje cell hamartoma) most of the surgically excised tumors are benign [1]. Since Miscellaneous hamartomas there is no counterpart tumor in other organs or lack of Tumors of fibrous tissue histogenetical and pathological evidence of true tumor for Fibroma papillary fibroelastoma, histiocytoid cardiomyopathy, Solitary fibrous tumor of pericardium lipomatous hypertrophy of the interatrial septum or cystic Benign fibrous histiocytoma tumor of atrioventricular node, and others, pathological Inflammatory pseudotumor classification is very difficult for these tumors [2]. And Vascular tumors and tumor-like lesions also, it seems difficult to classify the tumors by histological Varix type, because there are many tumors whose origin is not Hemangioma clear like tumors appear in other organs and tissues. A Hemangioendothelioma typical example is that in cardiac myxoma, origin of car- Hemangiopericytoma diac myxoma and tumor characteristics is not yet eluci- dated, and it contains various cell components like Lymphangioma epithelium, endothelium, nerve, and undifferentiated Tumors and proliferations of fat mesenchyma which potentially differentiate into many Lipomatous hypertrophy, interatrial septum tissues such as blood vessels, glandular structures, bones, Lipomatous hamartomas of cardiac valves and foci of extramedullary hematopoiesis [3]. Also, sar- Lipoma coma is a malignant cardiovascular tumor, and despite very The fatty heart low incidence, many types of sarcomas which found in Tumors and tumor-like lesions of mesothelial cells other organs were reported in the literatures [4]. However, Mesothelial cysts there is so-called undifferentiated sarcoma exists that Mesothelial/monocytic incidental cardiac excrescences cannot determine a certain tendency to differentiate into Mesothelial papilloma cells or tissues even if using the latest diagnostic technol- Tumors of neural tissue ogy such as immunohistochemistry, electron microscopy Granular cell tumor and genetic analysis [5]. Schwannoma/neurofibroma Because there are many cardiac tumors whose origin is Paraganglioma not yet determined and cell differentiation was not eluci- Tumors of smooth muscle dated, it is very difficult to systematically classify these Leiomyoma tumors. According to the well-known classification of Intravascular leiomyomatosis ‘‘tumors of the heart and great vessels’’ (Armed Forces Heterotopias and tumors of ectopic tissue Institute of Pathology: AFIP) published in 1996 [3] Bronchogenic/foregut cysts (Table 1), cardiac tumors including both cardiac tumors Tumors of the atrioventricular nodal region and pericardial tumors are classified into benign or Teratoma malignant cardiac tumors. Sarcomas of the aorta and pul- Ectopic thyroid monary artery, sarcomas of the inferior vena cava, and Intrapericardial thymoma leiomyomatosis of veins are classified in different catego- Malignant cardiac tumors ries. Benign cardiac tumors are further classified as tumors Sarcomas of unknown histogenesis, tumors of cardiac muscle, tumor Angiosarcoma of fibrous tissue, vascular tumors and tumor-like lesions, Malignant fibrous histiocytoma tumors and proliferations of fat, tumors and tumor-like Unclassified sarcoma lesions of mesothelial cells, tumors of neural tissue, tumors Myxosarcoma of smooth muscle, heterotopias, and tumors of ectopic Fibrosarcoma tissue. And malignant cardiac tumors are classified as Leiomyosarcoma sarcomas, malignant germ cell tumors, hematologic 123 Gen Thorac Cardiovasc Surg (2013) 61:435–447 437 Table 1 continued Table 2 WHO histological classification of tumors of the heart Rhabdomyosarcoma Benign tumors and tumor-like lesions Osteosarcoma Tumors of muscle cell differentiation Synovial sarcoma 1. Rhabdomyoma Malignant schwannoma (malignant peripheral nerve sheath 2. Histiocytoid cardiomyopathy/purkinje cell hamartoma tumor) 3. Hamartoma of mature cardiac myocytes Malignant mesenchymoma 4. Adult cellular rhabdomyoma Malignant hemangiopericytoma Pluripotent mesenchymal tumor Kaposi’s sarcoma 1. Cardiac myxoma Malignant germ cell tumors 2. Papillary fibroelastoma Hematologic tumors Haemangioma Lymphoma Tumors myofibroblastic cell differentiation Granulocytic sarcoma 1. Cardiac fibroma Mesothelial malignancies 2. Inflammatory myofibroblastic tumor/Inflammatory Malignant mesothelioma pseudotumor Metastatic tumors to the heart Cardiac lipoma Sarcomas of the aorta and pulmonary artery Cystic tumor of atrioventricular node Luminal (intimal) sarcoma Malignant tumors Unclassified sarcomas Cardiac sarcomas Malignant fibrous histiocytoma 1. Angiosarcoma Angiosarcoma 2. Epithelioid hemangioendothelioma Osteosarcoma 3. Malignant pleomorphic fibrous histiocytoma (MFH)/ Chondrosarcoma undifferentiated pleomorphic sarcoma Leiomyosarcoma 4. Fibrosarcoma and Myxoid fibrosarcoma Malignant mesenchymoma 5. Rhabdomyosarcoma Mural sarcomas 6. Leiomyosarcoma Leiomyosarcoma 7. Synovial sarcoma Angiosarcoma 8. Liposarcoma Malignant fibrous histiocytoma (MFH) Cardiac lymphoma Unclassified sarcomas Metastatic tumors to the heart Sarcomas of the inferior vena cava Pericardial tumors Mural leiomyosarcoma 1. Solitary fibrous tumor Luminal (intimal) sarcoma 2. Malignant mesothelioma Leiomyomas of veins 3. Germ cell tumors 4. Metastatic pericardial tumors Modified from reference [3] Modified from reference [4] WHO World Health

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