Angiosarcomas |
Poor prognosis, male > female |
Commonly affects right atrium or ventricle[22 Maleszewski JJ, Anavekar NS, Moynihan TJ, Klarich KW. Pathology, imaging, and treatment of cardiac tumours. Nat Rev Cardiol. 2017;14(9):536-49. doi:10.1038/nrcardio.2017.47. https://doi.org/10.1038/nrcardio.2017.47...
,33 Shapiro LM. Cardiac tumours: diagnosis and management. Heart. 2001;85(2):218-22. doi:10.1136/heart.85.2.218. https://doi.org/10.1136/heart.85.2.218...
,1919 Patel SD, Peterson A, Bartczak A, Lee S, Chojnowski S, Gajewski P, et al. Primary cardiac angiosarcoma - a review. Med Sci Monit. 2014;20:103-9. doi:10.12659/MSM.889875. https://doi.org/10.12659/MSM.889875...
]
|
Symptoms presented late and include right-sided heart failure, cardiogenic shock, pericardial effusions |
Highly aggressive, with high rate of recurrence; echocardiography is first line imaging; C-CT and C-MRI are often used in metastatic disease involvement[55 Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol). 2007;19(10):748-56. doi:10.1016/j.clon.2007.06.009. https://doi.org/10.1016/j.clon.2007.06.0...
,1919 Patel SD, Peterson A, Bartczak A, Lee S, Chojnowski S, Gajewski P, et al. Primary cardiac angiosarcoma - a review. Med Sci Monit. 2014;20:103-9. doi:10.12659/MSM.889875. https://doi.org/10.12659/MSM.889875...
,2828 Leduc C, Jenkins SM, Sukov WR, Rustin JG, Maleszewski JJ. Cardiac angiosarcoma: histopathologic, immunohistochemical, and cytogenetic analysis of 10 cases. Hum Pathol. 2017;60:199-207. doi:10.1016/j.humpath.2016.10.014. https://doi.org/10.1016/j.humpath.2016.1...
]
|
Treatment is often palliative rather than curative |
Rhabdomyosarcomas |
Commonly affects either ventricle[55 Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol). 2007;19(10):748-56. doi:10.1016/j.clon.2007.06.009. https://doi.org/10.1016/j.clon.2007.06.0...
]
|
Aggressive tumours with high rates of recurrence and metastatic involvement[55 Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol). 2007;19(10):748-56. doi:10.1016/j.clon.2007.06.009. https://doi.org/10.1016/j.clon.2007.06.0...
,1111 Hoffmeier A, Sindermann JR, Scheld HH, Martens S. Cardiac tumors -diagnosis and surgical treatment. Dtsch Arztebl Int. 2014;111(12):205-11. doi:10.3238/arztebl.2014.0205. https://doi.org/10.3238/arztebl.2014.020...
]
|
Similar clinical presentation to rhabdomyomas; symptoms include ventricular outflow obstruction, cardiac failure, and cardiogenic shock[44 Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol. 2005;6(4):219-28. doi:10.1016/S1470-2045(05)70093-0. https://doi.org/10.1016/S1470-2045(05)70...
,55 Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol). 2007;19(10):748-56. doi:10.1016/j.clon.2007.06.009. https://doi.org/10.1016/j.clon.2007.06.0...
]
|
Undifferentiated pleomorphic sarcomas |
Most common malignant type of cardiac tumour; no gender predisposition, with mean age at presentation » 45 years[22 Maleszewski JJ, Anavekar NS, Moynihan TJ, Klarich KW. Pathology, imaging, and treatment of cardiac tumours. Nat Rev Cardiol. 2017;14(9):536-49. doi:10.1038/nrcardio.2017.47. https://doi.org/10.1038/nrcardio.2017.47...
,2929 Orlandi A, Ferlosio A, Roselli M, Chiariello L, Spagnoli LG. Cardiac sarcomas: an update. J Thorac Oncol. 2010;5(9):1483-9. doi:10.1097/JTO.0b013e3181e59a91. https://doi.org/10.1097/JTO.0b013e3181e5...
]
|
Commonly found in the left atrium, generally systemic presentation including symptoms such as dyspnoea, chest pain, and cough[22 Maleszewski JJ, Anavekar NS, Moynihan TJ, Klarich KW. Pathology, imaging, and treatment of cardiac tumours. Nat Rev Cardiol. 2017;14(9):536-49. doi:10.1038/nrcardio.2017.47. https://doi.org/10.1038/nrcardio.2017.47...
,2929 Orlandi A, Ferlosio A, Roselli M, Chiariello L, Spagnoli LG. Cardiac sarcomas: an update. J Thorac Oncol. 2010;5(9):1483-9. doi:10.1097/JTO.0b013e3181e59a91. https://doi.org/10.1097/JTO.0b013e3181e5...
]
|
Surgical resection is treatment of choice given patient suitability for surgery |
Lymphoma |
This malignancy is typically confined to the pericardium with minimal extracardiac involvement |
Male > female predisposition, affected age » 60 years[3131 Pérez Baztarrica G, Nieva N, Gariglio L, Salvaggio F, Porcile R. Images in cardiovascular medicine. Primary cardiac lymphoma: a rare case of pulmonary tumor embolism. Circulation. 2010;121(20):2249-50. doi:10.1161/CIRCULATIONAHA.109.863126. https://doi.org/10.1161/CIRCULATIONAHA.1...
]
|
Increased incidence of lymphoma amongst immunosuppressed individuals (AIDS, HHV-8)[22 Maleszewski JJ, Anavekar NS, Moynihan TJ, Klarich KW. Pathology, imaging, and treatment of cardiac tumours. Nat Rev Cardiol. 2017;14(9):536-49. doi:10.1038/nrcardio.2017.47. https://doi.org/10.1038/nrcardio.2017.47...
,3131 Pérez Baztarrica G, Nieva N, Gariglio L, Salvaggio F, Porcile R. Images in cardiovascular medicine. Primary cardiac lymphoma: a rare case of pulmonary tumor embolism. Circulation. 2010;121(20):2249-50. doi:10.1161/CIRCULATIONAHA.109.863126. https://doi.org/10.1161/CIRCULATIONAHA.1...
]
|
Symptoms can either be systemic, including nights sweats, malaise, and pyrexia, or cardiospecific, such as atrial fibrillation, heart failure, or pericardial effusions[22 Maleszewski JJ, Anavekar NS, Moynihan TJ, Klarich KW. Pathology, imaging, and treatment of cardiac tumours. Nat Rev Cardiol. 2017;14(9):536-49. doi:10.1038/nrcardio.2017.47. https://doi.org/10.1038/nrcardio.2017.47...
]
|
Various treatment options, including chemo/radiotherapy, biological agents, such as monoclonal antibodies, and surgery (generally palliative intent)[22 Maleszewski JJ, Anavekar NS, Moynihan TJ, Klarich KW. Pathology, imaging, and treatment of cardiac tumours. Nat Rev Cardiol. 2017;14(9):536-49. doi:10.1038/nrcardio.2017.47. https://doi.org/10.1038/nrcardio.2017.47...
,44 Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol. 2005;6(4):219-28. doi:10.1016/S1470-2045(05)70093-0. https://doi.org/10.1016/S1470-2045(05)70...
,3131 Pérez Baztarrica G, Nieva N, Gariglio L, Salvaggio F, Porcile R. Images in cardiovascular medicine. Primary cardiac lymphoma: a rare case of pulmonary tumor embolism. Circulation. 2010;121(20):2249-50. doi:10.1161/CIRCULATIONAHA.109.863126. https://doi.org/10.1161/CIRCULATIONAHA.1...
]
|
Li-Fraumeni Syndrome |
Autosomal dominant inherited condition; associated with germline mutations of the TP53 gene |
Should be considered in young patients (< 45 years old), with recurrent cardiac tumour disease |
Increased predisposition of childhood cancers, most commonly associated malignancies, including sarcomas, brain tumours, breast cancer, leukemias, and adrenocortical cancers[3232 Varley JM. Germline TP53 mutations and Li-Fraumeni syndrome. Hum Mutat. 2003;21(3):313-20. Erratum in: Hum Mutat. 2003;21(5):551. doi:10.1002/humu.10185. https://doi.org/10.1002/humu.10185...
,3333 Masciari S, Dewanwala A, Stoffel EM, Lauwers GY, Zheng H, Achatz MI, et al. Gastric cancer in individuals with Li-Fraumeni syndrome. Genet Med. 2011;13(7):651-7. doi:10.1097/GIM.0b013e31821628b6. https://doi.org/10.1097/GIM.0b013e318216...
]
|