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A 14-Year Retrospective Review of Angiosarcoma: Clinical Characteristics, Prognostic Factors, and Treatment Outcomes with Surgery and Chemotherapy

Fury, Matthew G. MD, PhDa; Antonescu, Cristina R. MDb; Van Zee, Kimberly J. MDc; Brennan, Murray E MDc; Maki, Robert G. MD, PhDa

Original Article

PURPOSE Angiosarcoma is a rare vascular malignancy, and there are few published data to guide chemotherapy treatment decisions. We present a retrospective analysis of angiosarcoma encompassing all anatomic sites of disease presenting to a single institution over a 14-year period. Characteristics at presentation and prognostic factors are reviewed. For patients with unresectable disease, progression-free survival with various chemotherapy regimens is described.

PATIENTS AND METHODS Pathological confirmation of all cases was performed before they were included in this analysis. One hundred twenty-five patients with angiosarcoma were seen and treated between January 1, 1990 and December 31, 2003.

RESULTS Angiosarcoma showed marked variation by anatomic site regarding gender ratio, median age at diagnosis, overall survival, and response to chemotherapy. Overall 5-year survival was 31% for angiosarcoma. Superficial depth and negative microscopic surgical margins correlated with longer overall survival, but tumor size did not reach significance as a prognostic factor. For unresectable angiosarcoma, doxorubicin-based regimens yielded progression-free survival of 3.7–5.4 months. Paclitaxel achieved a progression-free survival of 6.8 months for scalp angiosarcoma and 2.8 months for sites below the clavicle.

DISCUSSION Angiosarcoma is an aggressive malignancy characterized by biologic heterogeneity at different anatomic sites and relative sensitivity to paclitaxel and doxorubicin.

aDepartment of Medicine of Memorial Sloan-Kettering Cancer Center, New York, New York.

bDepartment of Pathology of Memorial Sloan-Kettering Cancer Center, New York, New York.

cDepartment of Surgery of Memorial Sloan-Kettering Cancer Center, New York, New York.

Reprint requests: Robert G. Maki, MD, PhD, Memorial Sloan-Kettering Cancer Center, Department of Medicine, 1275 York Avenue, Howard 909, New York, New York 10021–6007. E-mail:

No benefits in any form have been or will be received from a commercial party related directly or indirectly to the subject of this article.

Dr. Fury is a fellow in medical oncology; all other authors are members of the attending physician staff.

These findings were presented in part at the Connective Tissue Oncology Society (CTOS) meeting, November 11–13, 2004, Montreal, PQ, Canada.

Received on January 6, 2005; accepted for publication February 15, 2005.

© 2005 Lippincott Williams & Wilkins, Inc.