An extremely aggressive sarcoma which typically presents with multifocal lesions in an anatomic region. This tumor may arise in association with a bone infarct or Paget's disease.
High grade angiosarcoma seems to have two distinct clinical presentations. First, the lesion can present as multiple lesions in a single bone, two or more adjacent bones, or perhaps all the bones of a limb. These lesions seem to have an indolent course and the prognosis remains good. The second presentation is that of single or multiple rapidly progressive lesions that metastasize to other bones or to the lung this form of the disease has a very poor prognosis. This case illustrated the later type.
Patients present with increasingly severe bone pain.
The radiographs shown here are of a 35 year old recently married auto mechanic whose wife had just had their first baby. He presented with severe pain in the distal femur. The initial work-up revealed multiple lesions in both lower extremities, including a lesion in the mid-diaphysis of the ipsilateral tibia and two lesions in the contralateral femur. A CT scan of the chest showed pulmonary nodules. Biopsy showed high-grade angiosarcoma of bone. Multiple, lytic, aggressive appearing lesions in a bone or adjacent bones.
Wide surgical resection and ajuvant therapy.
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