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Original Article |
1 Orthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital, Yawkey 7B-7926, 55 Fruit Street, Boston, Massachusetts 02114
2 Department of Radiation Oncology, Massachusetts General Hospital, Yawkey 7B-7926, 55 Fruit Street, Boston, Massachusetts 02114
3 Hematology/Oncology, Department of Medicine, Massachusetts General Hospital, Yawkey 7B-7926, 55 Fruit Street, Boston, Massachusetts 02114
4 Department of Pathology, Massachusetts General Hospital, Yawkey 7B-7926, 55 Fruit Street, Boston, Massachusetts 02114
5 Surgical Oncology, Department of Surgery, Massachusetts General Hospital, Yawkey 7B-7926, 55 Fruit Street, Boston, Massachusetts 02114
Correspondence: Address correspondence and reprint requests to: Sam S. Yoon, MD; E-mail: syoon{at}partners.org
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, 2291) 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.
Key Words: Sarcoma Angiosarcoma Outcome Radiotherapy
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