Massive osteolytic bone metastases from a primary aortic sarcoma: a case report

We present an unusual case of an aortic intimal sarcoma, which originally manifested itself by the presence of extensive radiologically osteolytic lesions in the long bones of the lower 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 diagnosis only came to light at autopsy. This revealed a tumour in the lower thoracic aorta which, unusually for aortic sarcoma, consisted of loosely packed bland spindle cells with no necrosis and infrequent mitoses. Immunocytochemistry was unhelpful but electron microscopy suggested 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 generally pleomorphic in appearance. Embolic phenomena and post mortem diagnosis are usual although occasional antemortem diagnosis has been made using computed tomography (CT) and magnetic resonance imaging (MRI) scanning with the latter being the investigation of choice.