Long-term survival of a patient with angiosarcoma treated with tailored paclitaxel

Abstract

Angiosarcomas are rare high-grade aggressive vascular malignancies of endothelial cell origin which account for approximately 1% of all soft tissue sarcomas and have a poor prognosis. The cornerstone of treatment is a large en bloc resection with negative margins, when feasible. However, the aggressive behavior, the diffuse pattern and the clinically undetectable spread of the disease makes a complete resection very difficult to achieve. At presentation, inoperable or metastatic disease is common. A phase II study has demonstrated clinical benefit and reasonable tolerance to treatment with paclitaxel. Case presentation: A 68 year old man presented with facial angiosarcoma. After a multidisciplinary evaluation, the disease was considered locally advanced and not amenable to curative-intent surgery. Weekly paclitaxel was chosen as first-line treatment, starting on October 2009. Partial response was achieved after approximately 2 months of chemotherapy. Ten months later systemic treatment was stopped and the patient was evaluated by the radiation oncologist, a total dose of 60 Gy was delivered. Two months later, a rapidly progressing cutaneous recurrence in the irradiated area with nasal bleeding was noted. Due to the previous prompt and excellent response, treatment with paclitaxel was resumed, using the same weekly schedule. Again, partial response was achieved. At that point, it was reasonable to plan a long-term maintenance treatment with tailored schedule; paclitaxel was administered every 2 weeks. After more than 4 years of maintenance therapy, with an interval between consecutive administrations of no longer than 2 weeks, the patient is still in almost complete remission.