Malignant Cardiac Tumors Rad-Path Correlation

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1 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%)

2 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

3 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)

4 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

5 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

6 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

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