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- 1. PHAM QUANG THIENPHU, MD 1
- 2. Classification Incidence Clinical manifestations and Diagnostic evaluations Approach to cardiac mass Benign tumors Primary maglinant tumors Secondary cardiac tumors Contents 2
- 3.
- 4. Nonneoplastic tumors: •Thrombi •Infections •Cysts varices •Degenerativeprocesses (calcification) •Heterotopias •Extracardiac processess (hiatal hernia, esophageal diverticulum) Classification 4 Chapter 4 - Cardiac tumors: Overview and Pathology Joerg Herrmann
- 5. Among theneoplastic lesions, benign and maglinant tumors can be distinguished as well as primary and metastatic processes. Fewer than 10% of primary cardiac neoplasms are maglinant. The classification of some lesions, such as papillary fibroelastoma, is jet unclear despite the fact that this is thought to be one of the most common adult cardiac tumors, and why precisely they arise is poorly understood. Classification 5 Chapter 4 - Cardiac tumors: Overview and Pathology Joerg Herrmann
- 6. WHO – classification(2015) 6
- 7. WHO – classification(2015) 7
- 8. WHO – classification(2015) 8
- 9. Pseudotumors Mural thrombi Heterotopiasand tumors of ectopic tissues Tumors of atrioventricular nodal region Teratoma Ectopic thyroid Tumors of mesenchymal tissue Harmatoma of endocardial tissue Papillary fibroelastoma Harmatoma of cardiac muscle Rhadomyoma Histocytoid cardiomyopathy (Purkinje cell) Classification by Pathology 9 Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann
- 10. Tumors and neoplasmsof fat Lipomatous hypertrophy, interarterial septum Lipoma Liposarcoma Tumors and neoplasms of fibrous and myofibroblastic tissue Fibroma Inflammatory pseudotumor (inflammatory myofibrolastic tumor Sarcomas (maglinant fibrous histocytoma, fibrosarcoma, leiomyosarcoma) Classification by Pathology 10 Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann
- 11. Vascular tumors and neoplasms Hemangioma Epitheliodhemangioendothelioma Angiosarcoma Neoplasm of uncertain histogenesis Myxoma Neoplasm of neural tissue Granular cell tumor Schwannorma/neurofibroma Classification by Pathology 11 Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann
- 12. Paragandolioma Maglinant schawannoma/ neurofbrosarcoma(rare) Maglinant lymphoma Maglinant mesothelioma Metastatic tumors to the heart Classification by Pathology 12 Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann
- 13. Primary cardiactumors are extremely rare. As an example, in one series of over 12,000 autopsies, only seven were identified, for an incidence of less than 0.1 percent. Metastatic involvement of the heart is over 20 times more common and has been reported in autopsy series in up to one in five patients dying of cancer. Incidence William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date 13
- 14. Why are cardiactumors rare ? 14 Matters of the Heart: Why Are Cardiac Tumors So Rare? – National Cancer Institute https://www.cancer.gov/types/metastatic-cancer/research/cardiac-tumors
- 15. Incidence 15 Clinical classification ofcardiovascular tumors and tumor-like lesions, and its incidences Jun Amano, Jun Nakayama, Yasuo Yoshimura, and Uichi Ikeda
- 16. Incidence of tumorsorginated from the heart 16
- 17. Incidence of tumorsorginated from the heart 17
- 18. Incidence of tumorsorginated from the heart in childen 18
- 19.
- 20. Clinical manifestation Cardiactumors: symptomatic or incidentally found Symptoms may mimic other cardiac conditions => clinical challenge is to consider the possibility of a cardiac tumor so that the appropriate diagnostic test(s) can be conducted. Echocardiography, magnetic resonance imaging (MRI), and/or computed tomography (CT). 20 William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 21. The specificsigns and symptoms of cardiac tumors generally are determined by the location of the tumor in the heart and not by its histopathology. Benign histology does not necessarily equate to a benign clinical course. Small, histologically benign lesions can still cause devastating consequences through thromboembolic events or arrhythmogenesis. Large, histologically benign lesions can obstruct blood flow and result in heart failure. Clinical manifestation 21 William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 22. Embolization: usuallysystemic but can be pulmonic. Aortic valve and left atrial tumors were associated with greatest risk of embolization. Obstruction of the circulation through the heart or heart valves, producing symptoms of heart failure. Interference with the heart valves, causing regurgitation Direct invasion of the myocardium, resulting in impaired left ventricular function, arrhythmias, heart block, or pericardial effusion with or without tamponade. Invasion of the adjacent lung may cause pulmonary symptoms and may mimic bronchogenic carcinoma. Constitutional or systemic symptoms Mechanism of symptoms production 22
- 23. Left atrial tumors Rightatrial tumors Right ventricular tumors Left ventricular tumors Clinical manifestration 23
- 24. Tumors arisingin the left atrium tend to grow into the atrial lumen and cause symptoms by obstructing blood flow or creating mitral regurgitation. => Mitral valve disease, heart failure and/or PAH Dyspnea, orthopnea, paroxysmal nocturnal dyspnea, pulmonary edema, cough, hemoptysis, edema, and fatigue. Left atrial tumors may release tumor fragments or thrombi into the systemic circulation => embolization and neurologic complications. Benign myxomas are the most common tumors arising in the left atrium . Left atrial tumors 24 William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 25. Tumors arisingin the right atrium grow into the atrial lumen and obstruct blood flow. => hemodynamic changes similar to tricuspid stenosis. Signs and symptoms of right heart failure. Tumor fragments may be released into the pulmonary circulation, causing symptoms consistent with pulmonary emboli. Right atrial hypertension can result in shunting of venous blood into the systemic circulation if a patent foramen ovale (or atrial septal defect) is present => hypoxemia or systemic emboli. Myxomas are the most common tumors of the right atrium. Sarcomas, angiosarcomas have been reported. Right atrial tumors 25
- 26. Lesions arisingin the right ventricle most commonly interfere with filling and/or outflow from the right ventricle => rightsided heart failure. Tumors arising in the right ventricle can be misdiagnosed as pulmonic stenosis, restrictive cardiomyopathy, or tricuspid regurgitation. Right ventricular tumors 26 William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 27. Tumors arisingin the left ventricle may be intramural and present with arrhythmias or conduction defects. Systemic embolization or outflow obstruction Syncope or left ventricular failure. Left ventricular tumors 27 William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 28.
- 29. Whether or nota cardiac tumor is present. The location of the lesion within the heart. Whether a tumor is benign or malignant (to extent possible). => Echocardiography, cardiac computed tomography (CT), cardiac magnetic resonance imaging (MRI) Diagnostic evaluation: Goals 29 Chapter 5 - Cardiac tumors: Imaging Joerg Herrmann
- 30. Widely available, simple, noninvasive technique for the initial evaluation. Images both the myocardium and the cardiac chambers => identify the presence of a mass and its mobility. Provide information about any obstruction to the circulation, as well as the likelihood that the tumor could be a source of emboli. Echocardiography 30 Chapter 5 - Cardiac tumors: Imaging Joerg Herrmann
- 31. Use ofintravenous ultrasound contrast agents can improve the diagnostic utility of echocardiography. Evaluate the vascularity and perfusion of cardiac masses Differentitate nonvasuclar thrombi from vascular neoplasms 3D echocardiography: may be benificial in selected cases. Routine use: uncertain benefit. Echocardiography 31 Chapter 5 - Cardiac tumors: Imaging Joerg Herrmann
- 32. More informative Proximity ofthe esophagus to the heart. The lack of intervening lung and bone. The ability to use highfrequency imaging transducers that afford superior spatial resolution. Transesophageal echocardiography (TEE) 32 Chapter 5 - Cardiac tumors: Imaging Joerg Herrmann
- 33. Fast imageacquisiton High spatial resolution of cardiac structures Compared with echocardiography, CT provides superior tissue characterization, specifically of fat and calcium. ECG-gated CT: functional analysis of cardiac valves and myocardial function as well as multiplanar reconstructions. Evaluation entire thorax Cardiac CT 33 Chapter 5 - Cardiac tumors: Imaging Joerg Herrmann
- 34. Best tissuecharacterization Provide functional evaluation of cardiac structures and morphologic evaluation in multiple planes Imaging the entire thorax. Cardiac MRI 34 Chapter 5 - Cardiac tumors: Imaging Joerg Herrmann
- 35. Modality Pros Cons EchocardiographyWidely available, highest spatial and temporal resolution, real-time evaluation of dynamic structures Tissue characterization is limited, most operator – dependent modality Limited evaluation of extracardiac intrathoracic structures ECG-gated CT Very fast image accquistion, excellent characterization of fat and calcium Evaluates entire throax Tissue characterization is worse than MRI Worse temporal resolution than echo Ionizing radiation. Cardiac MRI Best tissue characterization, allows for structural and functional resolution Evaluates entire thorax Worst spatial resolution Long image accquistion time Safety issues with some implanted devices Imaging modalities of cardiac tumors 35
- 36. Evaluating tumorspread throughout the body Useful in identifying cardiac involvement in patients with metastatic tumors, atrial myxoma, or lipomatous septal hypertrophy. PET scan 36 Chapter 5 - Cardiac tumors: Imaging Joerg Herrmann
- 37. Mapping theblood supply of tumors arising from the epicardial surfaces must be performed with coronary angiography. This information is vital to the success of excising such tumors. Significant involvement of coronary arteries with tumor may require resection and grafting of such arteries. For those with epicardial tumors, coronary angiography is specifically required to assess the location of the tumor’s nutrient vessels and any that are parasitized by the tumor Coronary angiography 37
- 38. Quite challengingbecause of location. Some tumors (particularly those arising from in the right heart) are amenable to endomyocardial sampling by wave of bioptome. Maglinant lesions that involve pericardium => cytologic analysis or pericardial fluid. In general, the information from noninvasive imaging is sufficient to make a decision regarding the need for surgery, where a definitive histologic diagnosis can be established. Tumors biopsy 38 William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 39.
- 40.
- 41. Diagnosis •Patient – specificcharacteristics (age and clinical context: fever, DVT,…) •Lesions – specific characteristics (shape, form, localization) •Differential diagnosis: normal variant, thrombus, vegetation Approach to cardiac mass 41 Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann
- 42. Evaluation of cardiacmass 42 Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann
- 43. Evaluation of cardiacmass focusing on histologic context 43 Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann
- 44. 1. Myxoma 2. Lipoma 3.Papillary fibroelastomas 4. Rhadomyoma 44
- 45. Myxoma 50% ofall benign cardiac tumors in adults and 15% of such tumors in children. Infancy: rare Sporadically and tend to be more common in women Occurene: between third and six decades of life. 94% tumors are solitary 75% occur in left atrium; 10 – 20%: right atrium Low recurrence rate 45 Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 46. DNA genotypeof sporadic myxomas is normal in 80% of patients. 5% of myxoma patients show a familial pattern of tumor development based on autosomal – dominant inheritance. 20% sporadic myxoma patients have abnormal DNA genotype chromosomal pattern. Genetic findings 46
- 47. Younger Male= female More often (22%) have multicentric tumors orinating from either the atrium or ventricle. Higher recurrence rate after surgical resection (21 – 67%) Associated conditions (20%): adrenocortical nodule hyperplasia, sertoli cell tumors of the testes, pituitary tumors, multiple myxoid breast fibroadenomas, cutaneous myomas and facial or labial pigmented spots Familial Myxoma 47
- 48. Composed ofscattered cells within a mucopolysaccharide stroma. The cells originate from a multipotent mesenchyme that is capable of neural and endothelial differentiation. Myxomas produce vascular endothelial growth factor (VEGF), which probably contributes to the induction of angiogenesis and the early stages of tumor growth. Histology 48
- 49. Typical myxomasare pedunculated and gelatinous in consistency The surface may be smooth, villous, or friable. Tumors vary widely in size, ranging from 1 to 15 cm in diameter, and weigh between 15 and 180 g. About 35 percent of myxomas are friable or villous, and these tend to present with emboli. Myxomas tend to grow into the overlying cardiac cavity rather than into the surrounding muocardium. Histology 49
- 50. 50 Histology Large polypoidmass with atrial septum attached, highlighted by the arrow. Note the focal hemorrhage. This is a typical myxoma.
- 51. 51 Histology Pathologic specimen demonstratinga large myxoma (M) in the left atrium (LA)
- 52. 52 Histology Atrial myxoma.Clusters and single polygonal cells with an irregular cytoplasm and dark nuclei can be seen. Note the background of the myxoid material.
- 53.
- 54. Intracardiac obstruction Congestive heart failure Signs of embolization Constitutional symptoms Fever Weight loss or fatigue Immunologic manifestration Myalgia Weakness Arthralgia Cardiac arrthymias and infections: less frenquently 54 Clinical Presentation
- 55. LA Positioneddyspnea Signs and symptoms of heart failure associated with left atrial and pulmonary venous pressure. Syncope: mitral stenosis or temporary occlusion of the mitral orifice RA: Right-sided heart failure Tricuspid valve stenosis Central cyanosis and paradoxic embolization (patent foramen ovale is present) Ventricular myxomas: ventricular outflow obstruction 55 Obstruction
- 56. Because mostmyxomas are left-sided, approximately 50% of embolic episodes affect the central nervous system, owing to both intracranial and extracranial vascular obstruction => intracranial aneurysm, seizures, hemiparesis and brain necrosis. Retinal artery embolization with visual loss occurs in some patients. Other sites of embolization: iliac, femoral arteries, renal and coronary arteries. Right – sided myxomasemboli mainly obstruct Pas => PAH and even death from acute obstruction. 56 Embolization
- 57. Rare complication Clinical picture of endocarditis infection Increases the likelyhood of systemic embolization 57 Infection
- 58. Nonspecific abnormalities:chamber enlargement, bundle-branch blocks, axis deviation, atrial fibrillation (20%) Most ECGs are not helpful in establishing a diagnosis 58 ECG
- 59. Most usefultest for the diagnosis and evaluation of myxoma. Sensitivity of 2D echocardiography is 100%. TEE: provide information concerning tumor size, location, mobility, and attachment. Detect tumors as small as 1 to 3mm in diameter. Before operation: to exclude the possibility of biatrial multiple tumors. Postoperative TEE: ensures a normal echocardiography before leaving the operation room. 59 Echocardiography
- 60.
- 61. Coronary angiographysi performed in myxomas patients more than 40 years of age to rule out significant coronary disease. 61 Coronary angiography
- 62. Neither CTor MRI is needed for atrial myxomas if an adequate echocardigraphy is available. Exception: occasional right atrial myxoma that extends into one or both caval or tricuspid orrifices. CT or MRI should be reserved for the situation in which the diagnosis or characterization of the tumor is unclear after complete echocardigraphic evalution 62 CT and MRI
- 63. Only effectivetherapeutic option Should not be delayed because of the risk of embolization or cardiovascular complications, including sudden death Operative mortality us related to advanced age or disability and comorbid conditions Removal of atrial myxomas carries an operative mortality of 5% or less. Excision of ventricular myxomas can carry a higher risk (approximately 10%) 63 Treatment: surgical resection William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 64. Patients areat risk for recurrence of the myxoma, which may occur in 2 to 5 percent of cases, or the development of additional lesions. Recurrence is more common in patients whose primary tumor was multicentric, younger patients, patients with a family history of myxoma. The disease –free interval averages about 4 years and can be as brief as 6 months. DNA testing of all patients with cardiac myxoma may prove to be the best predictor of the likelihood of recurence. 64 Recurences
- 65.
- 66. Well –encapsuled tumors consisting of mature fat cells. Occure anywhere: pericaridum, subendocardium, subepicardium and intra-atrial septum. Any age – no sex predilection Slow growing - no more than a few centimeters in size, lipomas as large as 4.8 kg have been reported. 66 Lipoma William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date
- 67. Asymptomatic andare discovered incidentally on routine echocardiography, or at surgery or autopsy. Symptoms, when present, are generally related to local tissue encroachment (arrhythmias, conduction block, sudden death). Valvular tumors can cause insufficiency and symptoms of heart failure. Pericardial lipomas are typically an incidental finding and clinically insignificant. Rarely, a pericardial lipoma can assume gigantic proportions and its appearance on a chest radiograph may be mistaken for a huge pericardial effusion or massive cardiomegaly. The diagnosis can be made with echocardiography and the distinctive fat pattern seen on MRI 67 Lipoma
- 68.
- 69.
- 70. Large tumorsthat produce severse symptoms should be resected. Smaller, asymptomatic tumors encountered unexpectedly during cardiac operation should be removed if excision can be performed without adding risk to prmary procedure. 70 Lipoma: treatment
- 71. The secondmost common primary cardiac tumor in adults. Arise characterically from cardiac valves or adjacent endocardium. Cytomegalovirus has been discovered in these tumors, suggesting the possibility of viral induction of the tumor and chronic endocarditis. Their appearance is often compared to sea anemones, with frondlike arms emanating from a stalked central core. AV and semilunar valves are affected with equal frequency. 71 Papillary fibroelastoma
- 72. 72 Papillary fibroelastoma removedfrom the aortic valve. Note the thin multiple fronds that resemble a sea anenome. Papillary fibroelastoma
- 73. 73Reprinted from Journalof the American College of Cardiology, volume 30, Klarich, KW, EnriquezSarano, M, Gura, GM, et al. Papillary fibroelastoma: Echocardiographic characteristics for diagnosis and pathogenesis correlation, p.784, Copyright 1997 with permission from the American College of Cardiology
- 74. Approximately 30%of papillary fibroelastomas were asymptomatic and diagnosed incidentally, either by echocardiography, at cardiac surgery, or at autopsy. Clinical features – Symptoms usually were caused by embolization, either of the tumor itself or thrombus. The most common clinical presentation was stroke or transient ischemic attack, followed by angina, myocardial infarction, sudden death, heart failure, syncope or presyncope, and systemic or pulmonary embolic events 74 Papillary fibroelastoma
- 75. While somerecommend surgery for all patients because of the risk of embolization and associated morbidity, others have suggested that careful observation is an acceptable option for asymptomatic patients, as long as the tumor remains small and nonmobile. Surgery is recommended for patients who have had embolic events or complications directly related to tumor mobility (eg, coronary ostial occlusion), and those with highly mobile or large (≥1 cm) tumors. Recurrence of cardiac papillary fibroelastoma following surgical resection has not been reported. 75 Papillary fibroelastoma
- 76. The mostfrequently occuring cardiac tumor in children. Presents during the first few days after birth. 80 - 90 percent are associated with tuberous sclerosis, a hereditary disorder characterized by harmatomas in various organs, epilepsy, mental deficiency and sebaceous adenomas. Multiple (90%), occur with approximately equal frequency in both ventricles, the atrium is involved in fewer than 30% of patients. Rhabdomyomas are usually found in the ventricular walls or on the atrioventricular valves. 76 Cardiac Rhadomyoma
- 77. Firm, gray,nodular and tend to project into the ventricular cavity. Microscopy: myocytes of twice the normal sized filled with glycogen, containing hyperchromatic nuclei and eosinophilic – staining cytoplasmic granules. 77 Cardiac Rhadomyoma
- 78. Clinical findingsmay mimic valvular or subvalvular stenosis. Arrhythmias, particularly ventricular tachycardia and sudden death, may be a presenting symptom. Atrial tumors may produce atrial arrhythmias. Rarely, no intramyocardial tumor is found in a patient with ventricular arrhythmias, and the site of rhabdomyoma is located by electrophysiologic study. The diagnosis is made by echocardiography. 78 Cardiac Rhadomyoma
- 79.
- 80. 80 Cardiac Rhadomyoma Rhabdomyoma HéctorGonzalo Quiroga Pacheco, MD. Centro Medico Profesional Rosancar. Barquisimeto. Estado Lara – Venezuela
- 81. 81 Cardiac Rhadomyoma Rhabdomyoma HéctorGonzalo Quiroga Pacheco, MD. Centro Medico Profesional Rosancar. Barquisimeto. Estado Lara – Venezuela
- 82. Early operationis recommeded in patients who do not have tuberous sclerosis before 1 year of age. Symptomatic tumors often are both multiple and extensive, particularly in patients with tuberous sclerosis, who, unfortunately, have a dismal long- term outlook. In such circumstances, surgery offers little benefit 82 Cardiac Rhadomyoma Chapter 6 - Cardiac tumors: Treatment Joerg Herrmann
- 83. Most rhabdomyomasregress spontaneously, and resection is usually not required unless a child is symptomatic (1) Tuberous sclerosis is associated with more than half of all cardiac rhabdomyomas. The indications for surgical intervention in patients with tuberous sclerosis are the same as those for patients without this disease (2) 83 Cardiac Rhadomyoma (1)William H Gaasch, MD, Thomas J Vander Salm, MD,Cardiac Tumors (2017) Up To Date (2) Surgical treatment for cardiac rhabdomyoma in children 1994
- 84. Tuberous sclerosiscomplex (TSC) is a genetic disorder affecting cellular differentiation, proliferation, and migration early in development, resulting in a variety of hamartomatous lesions that may affect virtually every organ system of the body. 84 Tuberous sclerosis Tuberous Sclerosis - Medscape
- 85. 85 Tuberous sclerosis: clinicalcriteria Updated diagnostic criteria for tuberous sclerosis complex 2012
- 86. 86 Tuberous sclerosis: clinicalcriteria Definite diagnosis: Two major features or one major feature with ≥2 minor features Possible diagnosis: Either one major feature or ≥2 minor features
- 87. 87 Three hypopigmented maculesthe lower back/upper buttocks.
- 88. 88 Facial angiofibromas. Ungualfibromas.
- 89. 89 (A)Fibrous plaque onface. (B) (B) Fibrous plaque on scalp.
- 90. 90 Shagreen patch ondorsolumbar area of back.
- 91. 91 Dental pits Intraoralfibromas (gingival and labial indicated by arrows)
- 92.
- 93. 75% primarymaglinant cardiac tumors are sarcomas. Sporadically – no inherited linkage Entire age spectrum, usually occur in adults more than 40 years Mc Allister’s survey: Angiosarcomas (31%) Rhabdomyosarcomas (21%) Maglinant mesotheliomas (15%) Fibrosarcomas (11%) 93 Sarcoma
- 94. The clinicalpresentation is largely determined by the location of the tumor, rather than its histopathology. Symptoms: congestive heart failure, pleuritic chest pain, malaism anorexia and weight lost. Some develop refractory arrhythmias, syncope, pericardial effusion and tamponade. The diagnostic approach relies upon echocardiography, MRI, and CT to define the presence of a tumor and its anatomic relationship to normal structures. 94 Sarcoma
- 95. Surgical resection Chemotherapy Radiotherapy Heart transplantation Cardiac autotransplantation Palliativetherapy 95 Treatment of primary maglinant tumor
- 96. Maglinant primarycadiac tumors may grow to a large size before detection Extensive myocardial involvement or location affecting the fibrous trigone of the heart Complete resection impossible Orthotopic cardiac transplantation: treatment option 96 Cardiac transplantation
- 97. Most ofpatients undergone chemotherapy and radiation prior to transplantation. In the largest series, results of cardiac transplantation in patients with malignant tumors (most of which were sarcomas) were evaluated in a review of 21 cases. Although mean survival was only 12 months, seven patients were free of recurrent malignancy at a mean follow up of 27 months. 97 Cardiac transplantation
- 98. An alternativetreatment, cardiac autotransplantation, has shown promise. In these cases, the heart is excised, the tumor is resected ex vivo, and the heart is reconstructed before being reimplanted. The advantage of this procedure is the increased ease with which major resection and reconstruction can be performed, while at the same time avoiding the need for antirejection treatment. Because of poor survival, lesions requring a pneumonectomy in addition to cardiac transplantation should be considered a contraindication to surgery. 98 Cardiac autotransplantation
- 99. In general,sarcomas proliferate rapidly, and cause death through widespread infiltration of the myocardium, obstruction of blood flow through the heart, and/or distant metastases. Although complete resection is the treatment of choice, most patients develop recurrent disease and die of their malignancy even if their tumor can be completely resected. The median survival is typically 6 to 12 months 99 Prognosis
- 100. Angiosarcomas arecomposed of malignant cells that form vascular channels. 2 – 3 times more common in men than in women. Predominant in the right side of the heart (80% RA) These tumors tend to be bulky and aggressively invade adjacent structures, including the great veins, tricuspid valve, RV free wall, interventricular septum, and right coronary artery. 100 Angiosarcoma
- 101. 101 Angiosarcoma Angiosarcoma of theright atrium. (A) Initial echocardiogram apical four- chamber view demonstrating pericardial tamponade and a suspected right atrial mass. (B) Operative photograph showing a tumor in the free wall of the right atrium. (C) High-power section showing a malignant spindle cell tumour with spaces filled with red blood cells, typical of angiosarcoma
- 102.
- 103. Most ofthese tumors have spread by the time of presentation, usually to the lung, liver and brain. Without resection, 90% of the patients are dead within 9 to 12 months of diagnosis despite radiation or chemotherapy. 103 Angiosarcoma
- 104.
- 105.
- 106. Tumor Total (No.) Cardiac (%) Pericardial (%) Leukemia 42053.9 22.4 Melanoma 59 34.0 23.7 Lung cancer 402 10.2 15.7 Sarcoma 207 9.2 9.2 Breast cancer 289 8.3 11.8 Esophageal cancer 65 7.7 7.7 Ovarian cancer 115 5.7 7.0 Kidney cancer 95 5.3 0.0 Gastric cancer 3.8 3.6 3.2 Prostate cancer 186 2.7 1.0 Colon cancer 214 0.9 2.8 Lymphoma 75 - 14.6 Metastatic cardiac disease Data from Perry MC. Cardiac metastasis. In: Kapoor AS, editor. Cancer and the heart; 1986 106
- 107.
- 108. Means ofspread: Hematogenous and ultimately via coronary arteries Lymphatic channels Direct extension from adjacent organs: lung, breast, esophageal, thymic tumors subdiaphragmatic vena cava Pericardium is involved most often by direct extension of thoracic cancer. Heart: hematologous and/or retrograde lymphatic metastasis Pathophysiology 108
- 109. Pathophysiology J Clin Pathol.2007 Jan; 60(1): 27–34. 109
- 110. Clinical symptomsin only about 10% of afflicted patients The most common symptom is pericardial effusion or cardiac tamponade. Occasionally, patients develop refractory arrhythmias or congestive heart failure. The specific symptoms will reflect the site of cardiac involvement, in a manner analogous to primary cardiac tumors. Clinical manifestration 110
- 111. The diagnosticevaluation is the same as that for primary cardiac tumors and relies upon echocardiography, magnetic resonance imaging (MRI), and computed tomography (CT) to ascertain the extent of cardiac involvement. Echocardiography is particularly useful for diagnosis of pericardial effusion, irregular pericardial thickening, or intracavity masses interfering with blood flow. Diagnostic evaluation 111
- 112. Aaron D. Goldberget al. Circulation. 2013;128:1790-1794 112
- 113. Limited torelief of recurrent pericardial effusions or, ocassionally, cardiac tamponade. Symptomatic palliation with minimal patient discomfort and hospital stay. Intracardiac obstruction by cardiac metastases may necessitate surgery, although outcomes may be poor if ventricular function has been irreversibly compromised (2) Treatment: surgical therapy (2) Tumors Metastatic to the Heart,Aaron D. Goldberg, Ron Blankstein, Robert F. Padera 113
- 114. Leukemia orlymphoma involving the heart, even with curative intent. Systemic chemotherapy 114
- 115.
- 116. Tumors involvingthe heart may cause symptoms by obstruction of circulation, interference with heart valves, direct invasion of the myocardium, invasion of adjacent lung, embolization, or by the production of constitutional symptoms. The signs and symptoms of a cardiac tumor are generally determined by the location of the tumor in the heart rather than by its histopathology 116 Summary
- 117. If acardiac tumor is suspected, imaging procedures are used to determine whether or not a mass is present and where the tumor is located within the heart. Echocardiography is the simplest technique for such evaluation; MRI and CT provide more detailed information. Tumors that occur from or invade the epicardial surface of the heart require coronary angiography preoperatively to define distortion of the coronary arteries and determine coronary blood supply of the tumor. 117 Summary
- 118. Over 75%of cardiac tumors are benign, and the majority of these are myxomas. About 80% of myxomas arise in the left atrium and most of the remainder are found in the right atrium. Myxomas are managed with prompt surgical resection because of the risk of embolization or other cardiovascular complications. 118 Summary
- 119. Primary malignanttumors of the heart are rare; most of these are sarcomas. Primary sarcomas arising in the heart generally are rapidly progressive and cause death through infiltration of the myocardium, by obstructing circulation, or by distant metastases. When feasible, treatment is surgical, although most of these tumors recur relatively rapidly. 119 Summary
Editor's Notes
- #7 Harmatoma: mô thừa dạng bướu
- #8 Synovial sarcoma: sarcoma dạng hoạt dịch Leiomysosarcoma: u cơ trơn
- #10 Papillary fibroelastoma: bướu sợi chun dạng nhú Histocytiod cardiomyopathy: loạn nhịp – rối loạn ty thể
- #23 Constitutional: trchung liên quan đến thể trạng
- #34 Temporal resolution: độ phân giải theo thời gian Spatial resolution: độ phân giải không gian
- #42 Vegetation: sùi
- #49 Multipotent: có thể phát triển thành nhiều loại tb
- #50 Pedunculated: có cuống, consistency: Friable: dễ vụn, villous: có lông nhung
- #53 Polygonal: nhiều cạnh
- #68 Distinctive: phân biệt đc
- #72 Sea anemones: hải quỳ biển Frond: lá dương xỉ Emanating: phát ra
- #98 pertaining to a tissue transplant grafted into its normal place in the body.
- #113 Imaging modalities for the identification and characterization of cardiac metastases. Top (A–C), Cardiac magnetic resonance in a patient with metastatic melanoma shows a 2×1-cm mass involving the myocardium of the distal anterolateral wall that displays increased signal intensity on both T1-weighted (T1W) imaging and T2-weighted (T2W) imaging. D, Echocardiography reveals a mass in the right atrium, found to be renal cell carcinoma from direct invasion via the inferior vena cava. E, Contrast-enhanced gated cardiac computed tomography (CT) demonstrates a large multilobed mass shown as filling defect in the right atrium and right ventricle. The mass was resected with a biopsy showing a lipoleiomyoma. LV indicates left ventricle; and SSFP, steady-state free precession.
- #116 Metastatic tumors involving the heart and pericardium. A, Photomicrograph of metastatic melanoma involving the myocardium resulting in destruction of cardiac myocytes. Hematoxylin and eosin stain, ×200 magnification. B, Photomicrograph of metastatic lung adenocarcinoma involving the epicardial lymphatics. Hematoxylin and eosin stain, ×100 magnification. C, Gross photograph of a metastatic renal collecting duct carcinoma filling the pericardial space and encasing the heart, resulting in constrictive physiology. D, Gross photograph of a T-cell lymphoma involving the myocardium in the area of the anterior right ventricle and anterior interventricular septum, resulting in arrhythmias. E, Gross photograph of a large endocardial metastasis from a malignant peripheral nerve sheath tumor involving the endocardium in a patient with neurofibromatosis type 1 filling the right ventricle and resulting in right ventricular outflow tract obstruction.