Bone and Soft Tissue Sarcomas

Annals of Surgical Oncology

, Volume 14, Issue 6, pp 1953-1967

First online:

Treatment and Outcome of 82 Patients with Angiosarcoma

  • John A. AbrahamAffiliated withOrthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital
  • , Francis J. HornicekAffiliated withOrthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital
  • , Adam M. KaufmanAffiliated withOrthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital
  • , David C. HarmonAffiliated withHematology/Oncology, Department of Medicine, Massachusetts General Hospital
  • , Dempsey S. SpringfieldAffiliated withOrthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital
  • , Kevin A. RaskinAffiliated withOrthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital
  • , Henry J. MankinAffiliated withOrthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital
  • , David G. KirschAffiliated withDepartment of Radiation Oncology, Massachusetts General Hospital
  • , Andrew E. RosenbergAffiliated withDepartment of Pathology, Massachusetts General Hospital
    • , G. Petur NielsenAffiliated withDepartment of Pathology, Massachusetts General Hospital
    • , Vikram DesphpandeAffiliated withDepartment of Pathology, Massachusetts General Hospital
    • , Herman D. SuitAffiliated withDepartment of Radiation Oncology, Massachusetts General Hospital
    • , Thomas F. DeLaneyAffiliated withDepartment of Radiation Oncology, Massachusetts General Hospital
    • , Sam S. YoonAffiliated withSurgical Oncology, Department of Surgery, Massachusetts General Hospital Email author 

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Abstract

Background

Angiosarcomas are an uncommon type of malignancy that are generally thought to behave usually in a locally aggressive fashion; they often metastasize to distant sites.

Methods

Patients with a diagnosis of angiosarcoma treated at our institution between 1980 and 2006 were analyzed for patient demographics, tumor characteristics, multimodality treatment, and outcomes.

Results

A total of 82 patients were divided into those with primary and advanced disease. Overall, the median age was 65 (range, 22–91) years, and 44% of patients were women. Median size of tumors was 3.8 cm, and 76% of tumors were intermediate or high grade. Tumors were located throughout the body: 32 cutaneous, 22 deep soft tissues or organs, 10 radiation or lymphedema field, 8 bone, and 7 nonirradiated breast. Of 46 patients with primary disease, all patients underwent surgical resection, 67% received radiotherapy, and 27% received chemotherapy. Five-year disease-specific survival was 60%, and negative prognostic factors included intermediate or high grade, and tumors arising in a radiated or lymphedema field. Of 36 patients with advanced disease, 36% underwent a palliative operation, 78% received radiation, and 58% received chemotherapy. Median survival was just 7.3 months, and cutaneous tumors predicted a better prognosis compared with other sites.

Conclusions

Primary angiosarcomas treated with aggressive surgical resection and the addition of radiation for close margins or worrisome pathologic features can result in long-term survival in most patients. The role of adjuvant chemotherapy is unclear. Patients with advanced disease have a poor prognosis, but there can be dramatic responses to chemotherapy in a minority of patients.

Keywords

Sarcoma Angiosarcoma Outcome Radiotherapy