Primary cardiac malignant fibrous histiocytoma is extremely rare and its pa
thophysiological characteristics remain largely unknown. We treated a femal
e patient with persistent fever and disseminated intravascular coagulation.
Since ultrasonic echocardiography revealed the presence of a cardiac tumor
and her serum interleukin-6 level was elevated, we speculated she had a ca
rdiac myxoma. Histological examination of the surgically resected specimen,
however, revealed that the tumor was malignant fibrous histiocytoma. Altho
ugh her disseminated intravascular coagulation and heart failure were trans
iently improved after operation, local recurrence and systemic metastasis o
ccurred and she died 7 weeks after operation. Using the autopsied specimen,
we examined whether the malignant fibrous histiocytoma constitutively synt
hesized interleukin-6. The interleukin-6 content in the tumor was high, con
sistent with interteukin-6 production by the tumor. This was confirmed by i
mmunohistochemical analysis. To our knowledge, this is the first report dem
onstrating interleukin-6 production by a cardiac malignant fibrous histiocy
toma.