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Cardiac tumors: A pictorial MRI guide for differential

diagnosis

Poster No.: C-1276


Congress: ECR 2013
Type: Educational Exhibit
Authors: G. Ironi, A. Esposito, P. MARRA, F. De Cobelli, A. Del Maschio;
Milan/IT
Keywords: Tissue characterisation, Neoplasia, Education and training,
Diagnostic procedure, Contrast agent-intravenous, MR, Oncology,
Cardiac
DOI: 10.1594/ecr2013/C-1276

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Learning objectives

Magnetic resonance imaging (MRI) is the technique of choice for the evaluation of cardiac
masses. In order to perform a correct diagnosis the radiologist needs to know aspects
concerning epidemiology, clinical presentation and specific radiological features of heart
tumors and pseudomasses.

Our aims are:

• Focus reader's attention on the rare problem of cardiac tumors


• Present MRI sequences used in our clinical practice for the best evaluation
and characterization of cardiac masses
• Show specific features of the most frequent heart neoplasms, using selected
images from our institution
• Provide a systematic guide that can help the radiologist in the differential
diagnosis

Background

1. Epidemiology

The frequency of cardiac tumors is low, but they cause significant morbidity and mortality.
Neoplastic heart masses can be divided into metastatic or primary tumors. Metastatic
involvement of the heart is 40 times more frequent than primary cardiac neoplasms and
is often underdiagnosed. [1] Non cardiac tumors may involve the heart and pericardium
by one of four pathways: retrograde lymphatic extension, hematogenous spread (eg.
melanoma), direct contiguous extension (eg. bronchogenic carcinomas) or transvenous
extension (eg. tumor from the kidney or liver). [1] Primary cardiac tumors are very
rare; 75% of these are benign while only one fourth are malignant. [2] The commonest
benign primary tumor is mixoma, while lipoma and fibroma occur less frequently. [3-5]
Primary malignant tumors of the heart are predominantly sarcomatous in nature: the most
common of these is angiosarcoma [6], followed by sarcomas with different mesenchymal
differentiation. Another malignancy is primary cardiac lymphoma which predominantly
occurs in immunocompromised subjects. [7]

2. Clinical aspects

Clinical manifestations of cardiac tumors depend on their size and location: some may
remain clinically silent with accidental diagnosis, large masses or intracavitary mobile

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tumors may result in intracardiac obstruction or systemic embolization and infiltrative or
intramural lesions frequently cause arrhythmias. [4]

3. MR versus other imaging modalities

Echocardiography: A variety of imaging modalities are available for the accurate


evaluation of cardiac masses. Transthoracic echocardiography often is the initial imaging
tool employed in assessment of suspected cardiac neoplasm because it is inexpensive,
rapidly performed and ubiquitous. Important limits of this technique are operator
experience, restricted field of view and difficulties in patients who have large body
habitus. Transesophageal echocardiography can provide a more detailed assessment
of small masses, masses within the atria and masses associated with valves, but this
is more expensive and less available than transthoracic echocardiography, with the
limitation of a relatively narrow field of view and of a higher discomfort for patients. Most
importantly, echocardiographic approach provides limited tissue characterization which
is a fundamental point for the comprehensive evaluation of cardiac masses.

Computed Tomography: Since the introduction of multi-detector scanners, CT has been


increasingly utilized for cardiac imaging. Its major advantages consist in high spatial
resolution, wide field of view, ability to detect small calcification inside cardiac masses
and excellent visualization of extracardiac anatomy. However, CT has an inferior low soft
tissue contrast resolution than MR and suffers from significant limitations inherent to cine
studies (less temporal resolution and higher radiation exposure).

Magnetic Resonance: MR imaging is becoming the modality of choice in evaluating


heart neoplasms because it allows accurate confirmation about the presence of a
lesion, localization, extension and overall allows tissue characterization. In addition
it is non invasive and offers direct multiplanar imaging, large field of view and high
spatial, contrast and temporal resolution. T1-weighted, T2-weighted, and gadolinium
enhanced sequences are used for anatomic definition and tissue characterization,
whereas cine stady-state free precession (SSFP) imaging is used to assess functional
effects. Perfusion study is used to provide additional information about lesion vascularity
and composition. Avid first pass enhancement is characteristic of highly vascular tumors,
such as angiosarcoma. Delayed enhancement reflects slow contrast medium uptake
and washout within areas of expanded interstitium, such as fibrous tissue. [4] Perfusion
analyses are also useful in the evaluation of metastatic heart disease. As the relative
contrast enhancement depends on the absolute uptake of the contrast media both by
the lesion and by the surrounding tissue, the post-contrast signal enhancement of a
metastasis may appear different in comparison to the primary tumor. For instance, a
cardiac metastasis from hepatocellular carcinoma (Figure 11), which is known to have
an early and strong enhancement, could present as a low-enhancing mass if compared
to the surrounding myocardium. For this reason it should be useful to assess tumor

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enhancement by signal/time curves, in order to obtain reliable information about tumor
vascularization and eventually to compare the vascular behavior of cardiac suspected
metastasis with the primary tumor.

A relative limitation of MR imaging is the low sensitivity in depicting calcification, so, in


some selected cases, it should be combined with CT.

4. Differentiating malignancies from benign tumors: general radiological features

Imaging evaluation of cardiac tumors is important not only for diagnosis but also for
determination of prognosis and in planning therapy, including surgical resection. [3] In
order to make a differential diagnosis it's important to consider several features such as
specific predilection for certain cardiac chambers or valves, tumor mobility, attachment
site and signal characteristics. Furthermore, it is pivotal to differentiate between benign
and malignant neoplasms because the clinical approach is completely different.

Features that suggest malignancy are [3, 8]:

• invasion of extra-cardiac structures


• involvement of more than one cardiac chamber
• involvement of the right side of the heart
• tissue inhomogeneity
• poor definition of borders
• greater than 5 cm diameter
• presence of pericardial or pleural effusion

In addition, as recently shown by Bauner et al., the enhancement curve after


administration of contrast material may provide other criteria for the evaluation of tumor
aggressiveness. [9] After a cardiac mass has been detected, imaging should be the first
step aiding the clinician to choose the best clinical behavior: radiological signs of non-
aggressiveness suggest that a watchful waiting approach or simple surgical resection
could be safe; in a small number of cases imaging is not diriment and a biopsy should be
performed. Patients with unresectable malignancies may be offered chemotherapy (eg
lymphoma) or palliation (large infiltrative angiosarcoma).

Imaging findings OR Procedure details

1. MR tumor protocol

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As a multiparametric technique MR allows different approaches for the characterization of
cardiac masses and the best cardiac MR protocol must be suited individually based on the
location and the extent of the tumor. If not contraindicated all the patients with a suspect of
heart neoplasm should be preferentially investegated with the intravenous administration
®
of a gadolinium-based contrast agent (gadobutrol, Gadovist , Bayer Healthcare Pharma
in our series). A first both morphologic and functional evaluation is achieved with cine
studies that rely on the acquisition of "bright blood" balanced SSFP sequences which
have both T1w and T2w effects. Cine SSFP sequences have high spatial and temporal
resolution and are particularly useful to evaluate the mobility of a lesion and its impact
on cardiac contractility; they also allow to depict eventual valve defects when the lesions
involve such structures.

Morphologic MRI provides a more accurate understanding of the size and the extent
of a lesion and relies on the variable acquisition of multiplanar "black blood" T1w, T2w
and/or PDw sequences usually preceded by single or multiple saturation pulses that
can suppress selectively blood or fat signal to better characterize the neoplastic tissue.
In particular T2w STIR sequences are sensitive to fluid and edema and can enhance
myocardial and pericardial cysts or ischemic area inside the myocardium. About ten
minutes after the intravenous administration of the contrast media other multiplanar IR
TFE T1w sequences are acquired in order to depict the eventual fibrotic degeneration of
the lesions, which is frequent in benign conditions such as fibromas, fibroelastomas and
rarely mixomas. These sequences rely on the principle of the late enhancement which is
due to a delayed wash out of gadolinium from a fibrotic tissue, that causes a prolonged
T1 shortening effect.

In addition to this morphologic and functional information, the execution of a perfusion


study, which is based on the rapid acquisition of 3D T1w sequences on multiple slices
early after contrast agent administration, offers information about tumor vascularization
and vascular infiltration.

We applied the previously described sequences on a 1.5 tesla MR scanner. All the images
were collected over a period of five years (from 2008 to 2012) from the archives of our
institution with the help of a highly experienced radiologist (A. Esposito).

2. Benign tumors

We propose schematic tables and peculiar images representative of the most frequent
cardiac neoplasms (tables 1-6, figures 1-6).

3. Malignancies

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Tables 7-12, figures 7-11.

4. Tumor mimics

Thrombus (Fig. 12)

It is the most common intracardiac mass mimicking a cardiac tumor. It is more frequently
located in the left atrium, commonly in the presence of atrial fibrillation, or in severely
dysfunctional left ventricles. It can also be found in the right side of the heart, especially
in the right atrium when there is a central venous catheter. The signal intensity
characteristics of thrombus are well shown in table 13. Note that a thrombus can assume
different aspects depending on its age and that, in most cases, does not enhance with
gadolinium contrast material. This lack of enhancement is very useful in differentiating it
from a myxoma, although an organized thrombus may show some surface enhancement.

Extracardiac masses:

• Pericardial cyst (Fig. 13): benign congenital lesion that arises from
pericardium and does not communicate with pericardial space. It is
usually found at the right cardio-phrenic angle, although it may occur
anywhere in the mediastinum. This well-defined lesion has the MR imaging
characteristics of simple fluid (hypointense on T1-weighted images and
hyperintense on T2-weighted images) and does not enhance after contrast
material administration.
• Bronchogenic cyst
• Intrathoracic neoplasms
• Gastrointestinal hernias

Normal cardiac structures:

• Crista terminalis of the right atrium (Fig. 14)


• Eustachian valve
• Prominent ventricular trabeculae
• Highly trabeculated atrial appendages
• Hypertrophy of papillary muscles

Images for this section:

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Table 1

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Fig. 1: A mixoma with atypical cardiac location. A two-chambers sBTFE cine sequence
(A, B) shows a well-defined hypointense mass of the right ventricle's conus arteriosus
that doesn't appear to significantly affect cardiac contractility during systole. The lesion
is isointense to myocardium on PDw imaging without signal loss when a fat-saturation
prepulse is applied: this can help to distinguish it from a lipoma (C). On (IR-TFE) late
enhancement imaging (D) the mass doesn't present residual contrast enhancement. This
benign neoplasm may just require an annual follow-up by echocardiography.

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Table 2

Fig. 2: Two simultaneous papillary fibroelastomas respectively arising from the anterior
edge of the tricuspid valve and from the free wall of the right atrium. A long-axis
four-chambers cine sequence (A) shows two hypointense intracavitary pedunculated
lesions in the right atrium. On late-enhancement imaging only the tumor of the atrial
wall can be distinguished as a hypointense nodule with no evidence of residual contrast
enhancement (B).

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Table 3

Fig. 3: A large fibroma of the interventricular septum markedly hypointense on cine


imaging: in A and B the diastolic and the systolic phases of the cardiac cycle are
respectively shown on a short axis two-chambers plane; the intramural bulky mass which
impresses both the right and the left ventricular cavities limits the contractility of the

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septum. However this benign tumor has a well-delimited non-infiltrative aspect. The
lesion shows typical very-low signal intensity on short-T1-inversion-recovery imaging (C)
and doesn't appear to have a significant contrast enhancement immediately after the
intravenous administration of gadobutrol (D). Due to the hypovascular and fibrotic nature
of this lesion the enhancement becomes more evident 10 minutes after the contrast agent
administration with a typical hyperintensity which shows up on late-enhancement imaging
(E-F).

Table 4

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Fig. 4: A hemangioma of the posterior wall of the right atrium which shows heterogeneous
hyperintensity on T2w (PDw) imaging (A-B respectively on a coronal and axial plane)
with higher signal intensity when a fat-saturation prepulse is applied (C, STIR). The
heterogeneity of the signal intensity is usually due to hemorrhagic foci that, alone, are
not predictive for malignancy. Furthermore the lesion is not associated with pericardial
effusion and doesn't seem to have an infiltrative behavior.

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Table 5

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Fig. 5: A lipoma arising from the interatrial septum with intracavitary extension into
the right atrium appears as a well-defined non infiltrative mass: the lesion which
is hyperintense on cine imaging (A-B) doesn't significantly affect atrial filling during
the cardiac cycle. The soft-tissue consistence of the mass is reflected by its shape
deformation during systole (A) and diastole (B). On a sagittal plane PDw imaging the
tumor shows high signal intensity (C) which is completely suppressed with a fat-saturation
sequence (D).

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Table 6

Fig. 6: A paraganglioma arising from the inferior wall of the left atrium here appears as
a broad-base mass with high signal intensity on sagittal plane T2w imaging both with (B)
and without (A) a fat-saturation prepulse. The tumor, which usually grows within the atrial
wall originates from paragangliar cells and may have an encapsulated or an infiltrative
aspect with heterogeneous signal intensity due to hemorrhagic and/or necrotic foci.

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Table 7

Table 8

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Table 9

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Fig. 7: A primary angiosarcoma of the free wall of the right atrium with metastatic spread
to the lung. On cine imaging (A) the mass has a heterogeneous iso- to hyperinetense
«cauliflower» aspect (due to the presence of hemorrhagic and necrotic foci) and tends
to infiltrate adjacent structures and the pericardium with subsequent pericardial effusion
that typically can be hemorrhagic, leading to cardiac tamponade. Due to its infiltrative
behavior, the malignancy impacts negatively on cardiac contractility. On T2w-STIR
images the mass shows a heterogeneous hyperintensity (B). On perfusion imaging (C)
the lesion rapidly and strongly enhances with a described «sunray» aspect due to the
filling of large vascular channels representative of tumoral angiogenesis. Image D shows
the peculiar aspect on CT-scan of lung metastasis from angiosarcoma: a hyperdense
hypervascular-hemorrhagic core is sorrounded by a ground-glass area.

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Table 10

Fig. 8: A pericardial mesothelioma with loculated pericardial effusion. PDw imaging


on axial (A) and coronal (B) planes shows a heterogeneous mostly isointense mass
which seems to spread through the pericardial sheets. On gadolinium-enhanced T1w
imaging on the axial plane (C) the hypointense effusion appears delimitated by thickened
pericardial sheets which have a nodular and irregular aspect with an avid contrast agent
uptake.

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Table 11

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Fig. 9: Morphologic and cine studies of a primary cardiac lymphoma originating from
the right atrio-ventricular sulcus: cine images are shown on a long-axis four-chambers
(A) and on a short-axis two-chambers (B) planes. The isointense mass has an invasive
aspect and is associated with a large pericardial effusion. However the lymphoma shows
a typical growth that respects other structures encountered: the right coronaric artery
(arrow head in image B) which is completely surrounded by the tumor doesn't seem to
be compressed or infiltrated by it. Axial T2w-STIR images are characterized by a slight
hyperintensity that diffusely infiltrate the myocardium. Notably, on perfusion imaging (C)
the mass doesn't show a significant contrast enhancement.

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Table 12

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Fig. 10: Images in the upper half of the figure show an example of trans-caval
metastatic spread of a hepatoma: the tumor has an heterogeneous signal intensity and
is predominantly hyperintense to the liver. On perfusion imaging (B) the mass shows a
discrete contrast enhancement. C and D images illustrate a metastatic involvement of
the left ventricle from a mammary angiosarcoma: this is a case of hematogenous spread.
On a long-axis two-chambers cine sequence (C) the tumor appears as an isointense
irregular-shaped mass with intracavitary growth. On perfusion imaging (D) the lesion
enhances less than the myocardium.

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Fig. 11: A and B show a hematogenous metastatic spread of colorectal cancer to the
antero-inferior wall of the right ventricle. On cine imaging (A) the slightly hyperintense
irregular-shaped mass shows an invasive behavior with involvement of the subepicardial
fat of the inferior wall of the ventricle. On perfusion imaging (B) the tumor strongly
enhances in the arterial phase. In the lower half of the figure there is an exemple of a
direct cardiac involvement from a squamocellular carcinoma of the lung. On cine imaging
(C) the tumor seems to invade through the pericardial sheets and the subepicardial fat
even if the myocardium doesn't appear infiltrated. On perfusion imaging (D) it is visible a
separation line between the heart and the strongly enhancing tumor.

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Table 13

Fig. 12: An apical thrombus of the left ventricle. A and B respectively show the diastolic
and the systolic phases of the cardiac cycle: the cine study is particularly useful as
it demonstrates that the intracavitary mass is adjacent to an hypo-akinetic area of
myocardium. Furthermore, late enhancement imaging (C) illustrates that ventricular wall
near the thrombus is thinned and fibrotic, features suggestive for myocardial infarction.

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Table 14

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Fig. 13: Pericardial cysts (A-B) should be primarly distinguished from loculated pericardial
or pleural effusion on the base of clinical history and other imaging findings. They
usually have an homogeneous slight hyperintensity on PDw imaging (A) and are
markedly hyperintense on T2w imaging and cine imaging (B). Benign pericardial cysts
are frequently uniloculated and located at the right cardiophrenic angle (B) with no effects
on cardiac contractility. They cannot always be differentiated from pleural cysts. Cysts
of the myocardium are very uncommon: C and D respectively show well-delimited high
signal intensities both on T2w imaging with fat-saturation (STIR) and on cine imaging.
This is an intramyocardial cyst of the lateral wall of the left ventricle associated with a
discrete pericardial effusion.

Fig. 14: This cine "bright blood" image illustrates a prominent crista terminalis protruding
into the right atrium: this structure, that physiologically varies in size and extent among
individuals, could be misdiagnosed at echocardiography.

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Conclusion

Imaging evaluation of cardiac tumors plays a fundamental role as allows specific tumor
characterization in many cases. When specific tumor characterization is not possible,
imaging is useful for the assessment of features that, in addition to clinical data (signs,
symptoms and age at presentation), can help in differentiating malignancies from benign
tumors. The evaluation of tumor aggressiveness through imaging modalities is very
important to formulate a prognostic assessment (as malignant tumors have a poor
prognosis while benign tumor generally have a favourable prognosis) and to plan the best
suited therapeutic strategy. Thanks to its ability to better characterize soft tissues MR is
even superior to echocardiography and CT and it should guide patient management.

References

1. Chiles C, Woodard PK, Gutierrez FR, et al. (2001) Metastatic involvement of the heart
and pericardium: CT and MR imaging. Radiographics 21: 439-449

2. Chu LC, Johnson PT, Halushka MK, et al. (2012) Multidetector CT of the heart:
spectrum of benign and malignant cardiac masses. Emerg.Radiol. 19: 415-428

3. Sparrow PJ, Kurian JB, Jones TR, et al. (2005) MR imaging of cardiac tumors.
Radiographics 25: 1255-1276

4. Randhawa K, Ganeshan A and Hoey ET. (2011) Magnetic resonance


imaging of cardiac tumors: part 1, sequences, protocols, and benign tumors.
Curr.Probl.Diagn.Radiol. 40: 158-168

5. Lam KY, Dickens P and Chan AC. (1993) Tumors of the heart. A 20-year experience
with a review of 12,485 consecutive autopsies. Arch.Pathol.Lab.Med. 117: 1027-1031

6. Best AK, Dobson RL and Ahmad AR. (2003) Best cases from the AFIP: cardiac
angiosarcoma. Radiographics 23 Spec No: S141-5

7. O'Sullivan PJ and Gladish GW. (2008) Cardiac tumors. Semin.Roentgenol. 43:


223-233

8. Syed IS, Feng D, Harris SR, et al. (2008) MR imaging of cardiac masses.
Magn.Reson.Imaging Clin.N.Am. 16: 137-64, vii

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9. Bauner KU, Sourbron S, Picciolo M, et al. (2012) MR first pass perfusion of benign and
malignant cardiac tumours-significant differences and diagnostic accuracy. Eur.Radiol.
22: 73-82

10. De Cobelli F, Esposito A, Mellone R, et al. (2005) Images in cardiovascular medicine.


Late enhancement of a left ventricular cardiac fibroma assessed with gadolinium-
enhanced cardiovascular magnetic resonance. Circulation 112: e242-3

Personal Information

Gabriele Ironi, Antonio Esposito, Paolo Marra, Francesco De Cobelli and Alessandro Del
Maschio;

- Experimental Imaging Center, San Raffaele Scientific Institute, Milan, Italy

- Department of Radiology, San Raffaele University Hospital, Milan, Italy

Correspondence to:

- Gabriele Ironi: g.ironi@studenti.unisr.it

- Antonio Esposito: esposito.antonio@unisr.it

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