We report on a 43-year-old man with a primary sarcoma of the liver. The pat
ient was admitted to the hospital for evaluation of dyspnea, abdominal pain
in the right upper quadrant, diarrheal and fever. Physical examination rev
ealed hepatomegaly. Increased laboratory values were found for gamma-GT, LD
H, CA 125, and NSE, but not for aspartate and alanine aminotransferase. Com
puted tomography presented a tumor in the right lobe of the liver. Venous c
avography revealed a caval turner thrombus reaching up to the right atrium.
Major liver resection combined with replacement of the vena cava inferior
was proposed, but before operation the patient complained about short ness
of breath. Spontenous fragmentation of the tumor thrombus with consecutive
embolization of the lungs was suspected. Despite lysis therapy the patient
died because of right ventricular failure. Autopsy revealed a tumor measuri
ng 8 cm in diameter: which was located in the right lobe of the liver and i
nvaded the inferior vena cava. Because of multiple tumor aggregates seen in
the left and right main pulmonary arteries acute tumor embolization of the
lungs was regarded as cause of death. Histologically the tumor was compose
d of bizarre giant cells surrounded hi irregular spindle cells. The positiv
e immunoreactivity pattern of the tumor cells for vimentin, lysozym, and CD
68 justified the diagnosis of a malignant fibrous his tiocytoma (MFH) of th
e liver.