A 49-year-old woman presented with a uterine tumour with solid and cys
tic parts, Extension of the tumour into the broad ligament was noted a
t the operation with wormlike plugs of tumour within the veins, The hi
stological diagnosis of intravenous leiomyomatosis was evident. The di
sease turned out to be far advanced: a long continuous intraluminal ma
ss extending from the iliac veins to the right ventricle made a second
operation with thoracotomy and laparotomy mandatory Postoperative fol
low-up showed so far, 1.5 years after operation, no evidence of recurr
ence, Intravenous leiomyomatosis is a very uncommon finding. Though it
is histologicaly a benign smooth-muscle tumour, the biological behavi
our with intravenous growth which may involve the right side of the he
art, is quite aggressive. About 100 cases were reported in the literat
ure; in no case could the diagnosis be made before surgery.