Successful treatment of an angiosarcoma of the nose with radiation therapy

Angiosarcoma is a rare, aggressive malignancy of endothelial cells lining blood vessels. It poses therapeutic challenges since there is no standard established treatment. It is typically treated with resection and wide-field postoperative radiation therapy. Chemotherapy and radiation therapy have also been reported as initial therapies. Regardless of the treatment rendered, the risk of local regional failure and distant relapse remains high for this disease. We present the case of a patient who developed a well-differentiated angiosarcoma of the nose with bilateral malar extension. No commonly associated risk factors such as lymphedema, prior radiotherapy or chronic venous ulceration were present. Given her age, pre-existing renal condition and preference not to receive chemotherapy, systemic therapy was not utilized. Surgery was also refused by the patient due to the projected cosmetic deficit. The patient was ultimately treated with definitive radiotherapy, utilizing electrons to the central face, differential thickness bolus, an intraoral stent, eye shields, an aquaplast mask for immobilization and a wax-coated lead shield over the face in order to limit penumbra of the radiation beam. Right and left anterior 6-MV photons were used to tangentially treat the bilateral malar region in order to extend the field edges. At the time of this report, the patient remains disease free at nearly 2.0 years after radiotherapy. To the best of our knowledge, this represents only the second case in the literature reporting radiotherapy as a single modality treatment that resulted in complete remission of an angiosarcoma of the face.