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PRIMARY CARDIAC LYMPHOMA
Authored By: Andrew Homb and Jonathan McConathy.
Patient: 31 year old male
History:

31 year-old man presenting to the emergency department with:

 

Cough and blood tinged sputum (2 weeks)

Dypsnea (1 week)

3 pillow orthopnea (1 week)

Paroxysmal nocturnal dyspnea

 

All of these symptoms have been intensifying over the past 2 days

 

Chest radiograph on presentation

 

Figure 1

 

Interpretation?

 

Echocardiogram after pericardial drainage

 

Figure 2: 4 Chamber

Figure 3: 4 Chamber Doppler (MV plane)

Figure 4: RVOT

Figure 5: RVOT Doppler

 

Interpretation?

 

What nuclear medicine study may be helpful? 

 

Figure 6

Figure 7

Figure 8

Figure 9

Figure 10

Figure 11

Figure 12

Figure 13

 

Interpretation and next step in management?

 

 

Image Size:[small][as-submitted]

Fig. 1
Frontal Chest Radiograph

Multimedia: 245546_2_submitted.avi
4 Chamber Echocardiogram

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4 Chamber Doppler Echocardiogram

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RVOT Echocardiogram

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RVOT Doppler Echocardiogram

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Rotating PET

Fig. 7
Coronal PET

Fig. 8
PET-CT

Fig. 9
PET-CT

Fig. 10
PET-CT

Fig. 11
PET-CT

Fig. 12
PET-CT
Image Size:[small][as-submitted]

Findings:

Frontal Chest Radiograph:

 

Marked enlargement of the cardiac silhouette.  If this finding is acute, these findings may be due to a pericardial effusion. Echocardiography can be performed for further evaluation.  Otherwise, consider ischemic cardiomyopathy, anomalous coronary artery from pulmonary artery, or adjacent mediastinal opacity (such pericardial cyst/lymph node/mass).

 

Echocardiogram:

 

Multiple intracardiac masses of different sizes and shapes in the right atrium, main pulmonary artery, left atrium, and superior left pulmonary vein.  The largest of these is in the left atrium, measures 3.5 cm x 3 cm, and prolapses into the left ventricle during systole.  A massive pericardial effusion is present without hemodynamic compromise. 

 

PET-CT:

RADIOPHARMACEUTICAL: 13.9 mCi F-18 Fluorodeoxyglucose (FDG) i.v.

There are abnormally hypermetabolic masses of varying shapes and sizes involving the right atrium, left atrium, left atrial appendage with extension to the left upper pulmonary vein, and main pulmonary artery. A moderate sized pericardial effusion is present and there is a single focus of increased activity in the anterior pericardial space adjacent to the sternum, at table position 690, which may represent extracardiac extension of one of the cardiac masses. No additional foci of abnormally increased FDG uptake noted throughout the rest of the body to suggest extrathoracic disease.

 

There is mildly increased uptake along the median sternotomy site, postsurgical in nature. Several mildly prominent abdominal para-aortic lymph nodes are present demonstrating FDG uptake less than blood pool. These lymph nodes are likely reactive in nature.

 

Additional findings on the noncontrast CT portion study include a large right pleural effusion and a small left pleural effusion. There is associated compressive atelectasis, right greater than left. Both pleural effusions do not demonstrate increased FDG uptake. A right subclavian approach port is in place with its tip near the cavoatrial junction. There is a small amount of air in the pericardial sac likely from recent pericardiocentesis. The gallbladder is moderately distended. Diffuse body wall edema is noted.

DDx:

Primary Cardiac Tumors (benign and malignant)

 

Myxomas

 

Sarcomas

 

Others (leiomyoma, rhabdomyoma, fibroma, lipoma)

 

Metastasis

 

Lymphoma (Primary or Systemic)

Diagnosis: Primary cardiac B-cell lymphoma
General Discussion:

Full History:

 

31-year-old man presenting with clinical symptoms of rapidly progressive heart failure.  Multiple large intercardiac masses of varying shapes and sizes are present on the echocardiograpm involving the right atrium, main pulmonary artery, left atrium, and left upper pulmonary vein. The largest of these masses prolapses into the left ventricle during diastole. Biopsy of a mass along the main pulmonary artery demonstrated B-cell lymphoma. The PET-CT is requested for initial staging.

 

Discussion: Primary Cardiac Lymphoma

 

Extremely rare clinical entity

 

1.3% of all cardiac tumors

 

Typically located in the RA, RV, and less often the LA.  Extracardiac disease has been noted.

 

Patients generally present with symptomatic pericardial effusions, refractory heart failure, or syncope but the range of sx is very broad.

 

Diffuse Large B-Cell morphology in ~ 80% of cases.

 

Diagnosis often reliant upon myocardial biopsy or cytologic assessment of pericardial fluid.

 

Ominous prognosis: half of patients do not survive to the initiation of chemotherapy.

 

Can involve the epicardium, myocardium, and endocardium.

 

 

Treatment

 

CHOP has been the mainstay of treatment.

 

Recently Rituximab has been added to CHOP.

 

Arrythmias have been associated with CHOP

 

High incidence of sudden cardiac death after treatment

 

Overall prognosis is poor (median survival 1 month without tx, 1 year with tx)

 

Heart transplantation and stem cell transplantation have been tried.

References: https://my.statdx.com
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Additional Details:

Case Number: 245546Owner(s): Andrew Homb and Jonathan McConathyLast Updated: 12-07-2011
Anatomy: Cardiopulmonary   Pathology: Neoplasm
Modality: Conventional Radiograph, US, PETAccess Level: Readable by all users, writable by NucMed Certifiers
Keywords: primary cardiac lymphoma

Case has been viewed 31 times.
Certified by Jonathan McConathy on 04-20-2011

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