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6.1 Introduction
Primary cardiac tumors are a rare cause of heart disease, being found in autopsic series with a mean frequency of 0.02%, corresponding to 200 tumors in 1 million autopsies . Three quarters of primary cardiac tumors are benign and the rest are malignant. Atrial myxoma, a benign neoplasm, is by &i the most common primary cardiac tumor, while the majority of primary cardiac malignancies are angiosarco- mas or rhabdomyosarcomas (Table 6.1). Cardiac metastases are 10 to 40 times more frequent than primary cardiac tumors, melanoma being the most common, although chnically unde- tected in a large percentage of cases. The diagnosis of a tumor involving the cardiovas- cular system in alive patients was difficult in the past due to the elusive character of cHnical signs and symptoms, their recognition being usually made at post-mortem examinations.
Cardiac tumors or masses are frequently an unsuspected finding in asymptomatic patients.
Their cHnical manifestations, when present, are related to their locahzation, size or behavior, causing pericardial efiiision (metastases, mesothehomas), compression of the cavities (cysts, thymomas, lymphomas), myocardial restriction (fibromas, hemangiomas, sarcomas), paroxistic pulmonary or central venous con- gestion (myxomas), or peripheral embohsm (thrombi, papillary fibroelastoma).
Although a first line diagnostic tool, echo- cardiography does have Hmitations: poor image quaHty in those cases with a difficult acoustic window, and limited field of view, especially in paracardiac masses. In the latter, the differential diagnosis is frequendy challenging as extrinsic cardiac compression may be due to many different causes ' , such as pleural efiission, hiatal hernia, or, simply, a chest wall mal- formation as in pectus excavatum. In all these situations, C M R is an excellent alternative imaging method (Figure 6.1). But also in the study of intracardiac masses C M R has become the preferred technique, due to its excellent image resolution and the possibility of obtain- ing angulated image planes, although in some aspects, as in the detection of calcifications, computed tomography is ahead . Provided the variety of techniques available, a knowledge of their relative merits in the diagnosis of cardiac
FRANCESC CARRERAS masses is important (Table 6.2).
6.2 Technical Aspects in the Evaluation of Masses with CMR
The excellent soft tissue definition that C M R provides allows the clear delineation of the myocardium, pericardium, paracardiac fat, vascular structures, and the lungs, and thus facilitates the identification and study of abnormal masses. The wide field of view permits unlimited observation of the extension and the anatomical relationships of cardiac and paracardiac masses with the great vessels and the bronchial tree, information that is espe-
cially useful to guide surgeons in the design of an appropriate therapeutical strategy ' .
To some extent, C M R allows some degree of tumor tissue characterization by a com- parative analysis of signal intensity character- istics on the images from the different sequences available (Table 6.3). Easy to char- acterize are those tumors with a high fat content, such as lipomas , which stand out for their bright signal intensity in T l w spin-echo (SE) sequences (Figure 6.2). Comparing the characteristics of the same image in T l w and T2w SE sequences, the composition of an
Table 6.1 Classification of the most frequent masses and cardiac tumors
Primary
Benign
Myxoma Pericardial cyst Lipoma Fibroelastoma Rhabdomyoma Fibroma Hemangioma
Malignant
Sarcoma Mesothelioma Lymphoma
Direct extension
Lung carcinoma Breast carcinoma Esophageal carcinoma Mediastinal tumors
Secondary
Venous extension
Renal cell carcinoma Adrenal carcinoma Hepatocellular carcinoma Thyroid carcinoma Carcinoma of the lung Sarcoma of the uterus
Metastatic extension
Melanoma Leukemia Lymphoma Genitourinary tract Gastrointestinal tract
FIGURE 6.1
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F. 6.1. Axial (left) and sagittal (right) images showing a large hiatal hernia (asterisks) compressing the posterior
aspect of the heart. LA: left atrium; RA: right atrium.
94
Table 6.2 Usefulness of different diagnostic tecfinlques for tfie study of cardiac tumors*
x-ray CT scan
Primary benign Myxoma Pericardial cyst Lipoma FIbroelastoma Rhabdomyoma Fibroma
Primary maiignant Sarcoma Mesothelioma Lymphoma Secondary tumors Direct proliferation Vein proliferation Metastases
+ ++
0 0 0 0
+ + ++
+
0
+
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Angiography
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0 0 0
+ + + + + ++
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+
Tiatinn- J I L alurauc i
Echocardiography
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+ +++
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++
++
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+++
+-1-
leafiil- J 1 1 L- nratarart Hiannnctii
CMR
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•* tact * MnHific Salcedo et al .
Table 6.3 Baslcal differential diagnosis of a cardiac mass according to signal intensity characteristics
• l-iigh on T1w SE: fat, effect of paramagnetic contrast, recent subacute hemorrhage (1 to 3 days)
• Intermediate on Tlw and high on T2w SE: tumor, thrombus, cyst with elevated protein content
• Low in T1w and very high on T2w SE: serous cyst
• Intermediate on GRE: tumor
• Low on GRE: Thrombus or a mass with a high iron content
eSiision or cyst can be assessed (Figure 6.3), although it should be noted that the signal of the hematic collections varies according to the degree of hemoglobin decomposition related to time (Table 6.4). This information is usefial for the approximate dating of the evolution time of an hematoma (Figure 6.4). Masses and tumors with a high iron content presents with an intermediate signal intensity on T l w SE and low signal intensity on gradient-echo (GRE) imaging. This characteristic is useful to difer- entiate a thrombus (Figure 6.5) from a mass of another origin. The presence of hemorrhage or necrosis within the tumor can characteristically alter its appearance (Figure 6.6). O n the other hand, intravenous administration of paramag- netic contrast agents permits to estimate the
degree of vascularization of the mass (Figures 6.6 and 6.7), this helping in the identification of intramyocardial masses .
A complete scanning protocol of cardiac and paracardiac masses should include axial, cor- onal and sagittal multisUce T l w and T 2 w SE or turbo SE images, a multislice axial T l w SE adquisition after administration of gadohnium- DTPA, and fmally a cine G R E sequence in the plane of the cardiac mass, which is very useful in assessing tumor motion.
In practice, the decision to use C M R for the evaluation of a cardiac or paracardiac mass is almost always based on a previous echocardio- gram in which the presence of an abnormal mass was suspected. Although C M R will always be helpful in assessing the location, anatomical relations and characterization of the lesion, in our experience, difierentiation between benign and mahgnant cardiac tumors by their signal characteristics is usually dis- appointing, as some studies have confirmed .
6.3 Malignant Primary Cardiac Tumors
Malignant neoplasms are classified by tissue
type as mesenchimal (sarcoma), lymphoid
(lymphoma), and mesotheUal (mesothe-
Table 6.4 Signal intensity (SI) of an hemorrhage related to time and to type of hemoglobin Condition
Acute (<24 hours)
Recent subacute (1-3 days) Late subacute (3-14 days) Chronic subacute (>14 days)
{Hemoglobin Oxyhemoglobin Deoxyhemoglobin Metahemoglobin Hemosiderin***
T1w SE SI Intermediate High*
Intermediate**
Low
T2w SE SI Intermediate Intermediate High Low
* Homogeneous signal
* * Heterogeneous signal (intermediate/high)
***The accumulation of hemosiderin produces a very low or even absent 81.