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Angiosarcoma

Summary

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.
Complete Information on this Tumor
Introduction and Definition: 

Angiosarcoma is an aggressive sarcoma of bone that is most common in the femur, tibia, and pelvis. It is a rare form of bone sarcoma.

The bony lesions of high grade angiosarcoma tend to be eccentric, purely lytic, metaphyseal and diaphyseal, with no visible matrix mineralization and focal destruction of the cortex. They have a very aggressive appearance but there is no periosteal reaction and very little soft-tissue extension, similar to what might be seen in metastatic lesions from lung. They tend to occur as multiple lesions in the same bone or contiguous bones, as shown in this case.

Symptoms and Presentation: 

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.

X-Ray Appearance and Advanced Imaging Findings: 
The bony lesions of high grade angiosarcoma tend to be eccentric, purely lytic, metaphyseal and diaphyseal, with no visible matrix mineralization and focal destruction of the cortex. They have a very aggressive appearance but there is no periosteal reaction and very little soft-tissue extension, similar to what might be seen in metastatic lesions from lung. They tend to occur as multiple lesions in the same bone or contiguous bones, as shown in this case.
Preferred Biopsy Technique for this Tumor: 
Open, incisional biopsy.
Treatment Options for this Tumor: 
Angiosarcoma is treated by wide surgical excision. This tumor is insensitive to radiation and may metastasize to lungs, lymph nodes and abdominal organs by both hematogenous and lymphatic routes. Chemotherapy is not used. Since there was already widespread metastasis at presentation, surgical removal of the tumor and limb sparing surgery was not a viable option. Treament included locked intramedullary rodding of the femur and tibia to prevent pathologic fracture. Radiation of the rodded bones and the opposite femur as well as adjuvant chemotherapy was given. A lesion appeared at the tip of the tibial rod just above the ankle, just outside the radiated field. Due to the risk of fracture at this site, the patient was given additional radiation to the area of recurrence. The tumor failed to show any significant response to chemotherapy and the patient had a rapily progressive course . He expired approximately one year from the time of initial diagnosis.
Preferred Margin for this Tumor: 
wide
Outcomes of Treatment and Prognosis: 
Prognosis is generally poor, but depends on the tumor behavior , location, extent, and response to treatment.
Special and Unusual Features: 
This tumor may arise in association with a bone infarct or Paget's disease.