A previously healthy 55-year old Caucasian
male presented with a two month history of flu-like
symptoms, dry cough, and progressive shortness
of breath. He also presented with a one and a half
month history of a slowly growing painless swelling
on the left side of his neck. Examination revealed
normal vital signs, an erythematous, firm but mobile
mass on the left side of the neck, and signs of a
right sided pleural effusion.Transesophageal echocardiography (TEE)
showed a 4.5 X 3 cm echodensity in the right ventricle.
Furthermore, a 3.5 X 3.5 cm sessile echodensity
was noted in the right atrium. The left and right
ventricles were normal in size and function with an
ejection fraction of 60%. There was trace circumferential
pericardial effusion.CT scanning of the neck displayed a fungating
and ulcerating soft tissue mass situated
at the dermal layer measuring 3.5 X 1.7 cm and
located above the level of the hyoid at the lower
end of the left parotid gland. CT of the chest revealed
an extensive bulky filling defect in the right
atrium, right ventricle, and outflow track at the level
of the pulmonary valve. Multiple low density lesions
were evident in the right lobe of the liver (Figure 1).
Lymphadenopathy was noted inferior to the aortic
bifurcation.
Figure 1:
CT image showing multiple low density lesions in the
liver.
CT image showing multiple low density lesions in the
liver.In addition, biopsy of the neck mass revealed
a lymphoid infiltrate with atypical features.
A majority of cells were positive for CD20, CD10,
CD79a, and TdT, but negative for CD34, and BCL-2
on immunohistochemical staining. Proliferation rate approached 100% for Ki-67. The morphology was
consistent with a high grade B-cell lymphoma with
atypical Burkitt/Burkitt-like features.
DISCUSSION
Incidence of cardiac neoplasia is very low
(1). Most cardiac neoplasms are metastatic, atrial
myxoma being the most common primary cardiac
tumor. Lymphomas constitute 9% of the total metastases.
Cardiac involvement by disseminated
non-Hodgkin's lymphoma has been documented in
approximately 20% of autopsy cases to the heart
(2).Burkitt-like lymphoma is a highly aggressive
form of non-Hodgkin's lymphoma and associated
with poor short-term survival. Extranodal
Burkitt Lymphoma involving the heart is rare and
seldom recognized clinically. Diagnosis is usually made late because of the non-specific nature of
the symptoms. Right-sided heart failure, dyspnea,
superior vena cava obstruction, pericardial effusion
with or without tamponade, and rhythm disturbances
are the usual presenting features. The average
age at presentation is sixty, with a slight male predominance
(3). Transesophageal echocardiography
(TEE) is superior to transthoracic echocardiography
(TTE) in assessment of cardiac lymphomas
and typically shows a hypoechoic mass in the atria
or ventricles that is often associated with pericardial
effusion. Although multidetector computed tomography
and magnetic resonance imaging will provide
information about the staging of disease, biopsy
of the mass is needed to make the diagnosis (4).
Chemotherapy is the first line treatment, however
complete remission is achieved in less than 60% of
patients.
Figure 1: