We present an unusual case of an aortic intimal sarcoma, which origina
lly manifested itself by the presence of extensive radiologically oste
olytic lesions in the long bones of the low er Limbs. The histology of
these was puzzling and was first considered to represent a low grade
sarcoma of vasoformative tissue and subsequently skeletal angiomatosis
. Despite a good initial clinical response to disodium etidronate, the
patient ultimately developed small bowel infarction and the true diag
nosis only came to light at autopsy. This revealed a tumour in the low
er thoracic aorta which, unusually for aortic sarcoma, consisted of lo
osely packed bland spindle cells with no necrosis and infrequent mitos
es. Immunocytochemistry was unhelpful but electron microscopy suggeste
d myofibroblastic differentiation. The majority of previous reports of
the tumour in the literature lack information on electron microscopy
and immunocytochemistry and have suggested that these tumours are gene
rally pleomorphic in appearance. Embolic phenomena and post mortem dia
gnosis are usual although occasional antemortem diagnosis has been mad
e using computed tomography (CT) and magnetic resonance imaging (MRI)
scanning with the latter being the investigation of choice. Copyright
(C) 1997 by W.B. Saunders Company.