Angiosarcoma (bone)

Angiosarcoma of bone is a malignant vascular bone tumor. These are rare and account for <1% of malignant bone tumors. The majority of these tumors arising in bone are primary; however, a tiny percentage are either radiation-induced or associated with bone infarction.

Epidemiology

Most common in young individuals. Males are more commonly affected than females. 

Clinical presentation

Although there is no particular clinical presentation, the majority of the patients present with chronic dull pain and/or tumor mass. Other presentations include:

• associated neurological deficit

• pathological fracture (rare)

Pathology

Angiosarcomas can be unifocal or multifocal, with one study citing that up to one-third can be multifocal ⁴. The most common sites are short and long tubular bones of extremities ²:

• femur: 23%

• tibia: 18%

• humerus: 13%

• pelvis: 7%

Radiographic features 

Plain radiograph

They are usually purely lytic, eccentric and occur in a metadiaphyseal location. They can have a very aggressive appearance, without a periosteal reaction and less soft tissue extension. Can occur as multiple lesions in same bone. Metastatic lesions to other skeletal sites may be seen.

Treatment and prognosis

In general very poor prognosis and depends on location and extent. Treatment options include:

• wide surgical excision 

• limb-sparing surgery

• radiotherapy

Differential diagnosis

Possible differential considerations include:

• skeletal metastases

• other malignant vascular tumors of bone

  • hemangiopericytoma of bone: not possible to differentiate radiologically ²

  • hemangioendothelioma of bone: not possible to differentiate radiologically ²

See also

• angiosarcoma

  • musculoskeletal angiosarcoma