Treatment and outcome of 82 patients with angiosarcoma.
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.
Abraham
John A
JA
Orthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital, Yawkey 7B-7926, 55 Fruit Street, Boston, Massachusetts 02114, USA.
Hornicek
Francis J
FJ
Kaufman
Adam M
AM
Harmon
David C
DC
Springfield
Dempsey S
DS
Raskin
Kevin A
KA
Mankin
Henry J
HJ
Kirsch
David G
DG
Rosenberg
Andrew E
AE
Nielsen
G Petur
GP
Desphpande
Vikram
V
Suit
Herman D
HD
DeLaney
Thomas F
TF
Yoon
Sam S
SS
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001859:Bone Neoplasms / Q000473:pathology / Q000601:surgery; D001943:Breast Neoplasms / Q000473:pathology / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006394:Hemangiosarcoma / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D009381:Neoplasms, Radiation-Induced / Q000473:pathology / Q000601:surgery; D010166:Palliative Care; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000473:pathology / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000473:pathology / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome