Angiosarcoma of the scalp: What is the optimum management?

Abstract

Scalp angiosarcoma is a rare malignancy with a poor outcome. In the absence of randomized trials, the approach to management depends on retrospective series and case reports. Here, we report a 56-year-old male diagnosed initially with squamous cell carcinoma of the scalp at an outside center. When he underwent re-excision for recurrence in our center, histopathology showed a high-grade angiosarcoma. He was offered postoperative radiotherapy for the primary site. During the treatment, he developed preauricular nodal recurrence, which was also treated with radiotherapy. After radiotherapy, he had persistent preauricular nodes, for which single-agent chemotherapy was given, and the nodes were regressed. However, the recurrence of preauricular nodes was noted at the next follow-up visit, and he was re-evaluated with a positron emission tomography-computed tomography scan, which showed an extensive lung and nodal metastases. He was started on metronomic chemotherapy and his disease was stable at the time of the last follow-up. This case raises several questions, including whether electively addressing the regional nodes in the scalp angiosarcoma could improve the outcomes, whether higher doses of radiotherapy are beneficial or only add to the toxicity of treatment, the role that chemotherapy plays and the most appropriate regimen. Although some relevant studies have been discussed in brief, more evidence is needed to answer these questions.