CARDIOVASCULAR JOURNAL OF AFRICA • Vol 22, No 2, March/April 2011
AFRICA
97
ascites. Computed axial tomography confirmed the presence
of cervical and mediastinal lymphadenopathy which was most
prominent on the right side, but there was no increase in size of
the lymph nodes below the diaphragm.
The histological examination of material obtained by fine-
needle aspiration biopsy over the cervical nodes revealed signs
of Burkitt-like lymphoma, namely greater nuclear pleomorphism
and smaller numbers of prominent nucleoli than would be
expected in a classic Burkitt’s lymphoma.
The child was considered to have Burkitt-like lymphoma in
stage II-B. Diuretic therapy was stopped, and the patient under-
went chemotherapy with the CHOP regimen using ciclophosfa-
mide (600 mg/m
2
), vincristine (1.4 mg/m
2
) and doxorubicin (50
mg/m
2
) IV on the first day, and prednisone (60 mg/m
2
) orally
for five days. His clinical condition dramatically improved, with
remission of the signs of superior vena cava syndrome, reduc-
tion of the size of the intracardiac masses and remission of the
biological abnormalities. The patient was discharged after three
weeks but, unfortunately, was lost for follow up.
Discussion
Secondary cardiac tumours are mostly epicardial and asympto-
matic,
1,2
usually being seen in the setting of widely disseminated
malignancy. Less than 5% correspond to lymphomas, which
may be particularly difficult to diagnose as the cause of refrac-
tory heart failure. Although recent developments in imaging
techniques have contributed to progress in
in vivo
diagnosis of
cardiac tumours, a high index of suspicion is usually needed.
Endemic Burkitt’s lymphoma occurs in African children,
usually four to seven years old, involving the bones of the jaw
(and other facial bones), kidneys, gastrointestinal tract, ovaries,
breast, and other extra-nodal sites; lymph node involvement is
less common in this age group.
3
The differential diagnosis of
Burkitt’s lymphoma is broad, and precise diagnosis based on
histological, immunophenotypic and genetic features remains
the critical first step in planning appropriate therapy.
4
Although
excision biopsy remains the preferred method for histological
diagnosis of lymphoma, diagnosis and typing of lymphomas can
be achieved by fine-needle aspiration cytology of subcutaneous
lymph nodes,
5
a method frequently used in African settings.
Burkitt-like lymphoma is a highly aggressive B-cell lympho-
ma with a high proliferative rate, and histopathological charac-
teristics considered borderline between those of classic Burkitt
lymphoma and diffuse large B-cell lymphoma.
4,6
Extensive
cardiac involvement of Burkitt-like lymphoma in the absence of
HIV infection is rare and poorly documented in the literature.
7
Its outcome in childhood has improved with the use of short and
intensive B-cell non-Hodgkin’s lymphoma-directed therapy.
4,8
Conclusion
The case we report on highlights the need to consider the diag-
nosis of Burkitt’s lymphoma in sub-Saharan African children
presenting with heart failure and intracardiac masses, even in the
absence of HIV infection. It also shows that an improvement in
the education of both health personal and the parents is needed
to allow early diagnosis and adequate management of this condi-
tion, in order to improve its prognosis.
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Fig. 2. Echocardiography. A. Four-chamber view showing dilatation of the right atrium, which contains a large mass
attached to the interatrial septum, partially occluding the flow through the tricuspid valve. Notice the presence of
moderate pericardial effusion. B. The short-axis view, at the level of the aortic valve, shows thickening of the aortic
cusps and the tissue surrounding the valve. The right atrial mass is also seen attached to a thickened interatrial
septum.
A
B
