Malignant Cardiac Tumors Rad-Path Correlation Vincent B. Ho, M.D., M.B.A. 1 Jean Jeudy, M.D. 2 Aletta Ann Frazier, M.D. 2 1 Uniformed Services University of the Health Sciences 2 University of Maryland Malignant Cardiac Tumors Metastatic Disease to the Heart 12% of autopsies with widespread malignancy 20-40x more common than primary cardiac neoplasms Example: Lung Cancer, Lymphoma Tumor-like lesions Thrombus Valvular vegetations Primary Cardiac Tumors Malignant Neoplasms Sarcoma (2 nd most common primary cardiac neoplasm; 10-25%): Osteosarcoma Rhabdomyosarcoma Lymphoma (RARE as a primary) Role of Imaging Location, size, number, shape, mobility Tumor extent (myocardium, valve, pericardium, lung parenchyma) Tissue characterization Determine extra-cardiac involvement Surgical planning (surgical candidate?) Clinical Presentation Clinical presentation highly variable and depends on the mass s: Location Size Growth rate Friability (likelihood for embolus) Invasiveness Cardiac Sarcomas Most common primary malignant cardiac neoplasms Most common cell types: (37% of cases) Unclassified/undifferentiated sarcoma (24%) Malignant Fibrous Histiocytoma (MFH) (11% 24%) Leiomyosarcoma (8% 9%) Osteosarcoma (3% 9%)
Cardiac Sarcomas Primary cardiac sarcomas are highly aggressive lesions Uniformly fatal Poor survival (mean ~ 3 months to 1 year) Even after complete tumor excision, local recurrence and metastatic disease occur frequently and early usually within 1 year Largest group of differentiated cardiac sarcomas F=M, 20-50yo Arise in myocardium (90% RA) Irregular vascular channels containing RBCs Grossly hemorrhagic Pericardial invasion Chamber impingement Complications Tamponade Dysrhythmias Myocardial rupture Mass arising in RA wall Heterogeneous (hemorrhagic) Malignant pericardial effusion Pulmonary mets (30%) Originates in RA wall Heterogeneous signal (T1, T2) Nodular areas of increased intensity thrombus, hemorrhage +/- Pericardial thickening, nodules
Osteosarcoma Pre-Gd T1W Image Heterogeneous RA mass Foci of hemorrhage Post-Gd T1W Image Heterogeneous enhancement Non-enhancing necrotic regions Rare Almost exclusively LA Calcium (+/-) Tend to invade pulmonary vein Metastases often at presentation (lung, lymph nodes, thyroid, skin) Pre-Gd T1W Image Iso-intense to myocardium Invades Pulmonary Veins Osteosarcoma Post-Gd T1W Image Heterogeneous enhancement (necrosis) Rhabdomyosarcoma Most common primary cardiac malignancy of childhood No specific chamber predilection Arise from myocardium, more likely to affect valves than other sarcomas 2 types: Embryonal (more common; child or adults) Pleomorphic (less common, adults) Local recurrence common Myocardium, pericardium Rhabdomyosarcoma Typically non-hodgkin type Seen with greater frequency in immunocompromised patients, particularly in association with AIDS Note: Secondary Lymphoma much more common than primary (2nd most common met to heart; 16-28% pts with disseminated lymphoma have cardiac mets)
Histo: lymphoma cells surrounding, infiltrating residual myocytes (arrow) Majority are B-cell neoplasms Pericardial invasion typical Cytology diagnostic 67% Mural mass in RA>RV>LV>LA Multichamber 75% Intramural mass Chamber impingement SVC, IVC occlusion May be hypointense on T1W and T2W Variable enhancement patterns with Gd-DTPA
Metastatic Tumors 1) Lung Cancer 2) Lymphoma Leukemia Melanoma Extracardiac sarcoma Breast Cancer Renal Cancer Thyroid Cancer A Simple Approach to Cardiac Tumors Overall, most primary cardiac tumors are BENIGN. Myxoma Lipoma Fibroma The common primary cardiac MALIGNANCIES are: (adults) Rhabdomyosarcoma (children) Most malignant cardiac tumors are METASTATIC. Primary Cardiac Tumors Myxoma: Atrial (LA/atrial septum), endocavitary Papillary fibroelastoma: valvular Lipoma: fat sat MR confirms Rhabdomyosarcoma: Children, young adults : RA and aggressive Osteosarcoma: LA Lymphoma: Right heart + Pericardial effusion Features of Malignant Cardiac Tumors Intramural location or infiltration* Pericardial invasion/effusion Tumor necrosis Vascular invasion (pulmonary veins, SVC) Mediastinal invasion Pulmonary metastases * vs. intracavitary location or extension (occurs in both myxomas and sarcomas, thus not a helpful distinguishing feature) Cardiac Tumors Clinicopathological Features Benign AND malignant tumors may produce: Embolization (pulmonary, coronary and peripheral-esp CNS) Arrhythmias Obstructive symptoms (mass effect, valvular occlusion) Surgical resection may be indicated for either malignant and non-malignant lesions Cardiac Tumors Clinicopathological Features Best prognosis Congenital benign tumors (lipoma, rhabdomyoma, hemangioma, ventricular fibroma) Poorest prognosis Metastatic disease to the heart Primary cardiac sarcomas
Presentation Objectives At the end of this presentation, the participant will be able to: List types of cardiac tumors Discuss various characteristics of benign primary cardiac neoplasms Discuss various characteristics of malignant primary cardiac neoplasms