Major response to paclitaxel combined with irradiation as second-line therapy in metastatic angiosarcoma of the face and scalp

Conflicts of interest: none declared. Sir, Angiosarcoma (AS) of the face and scalp forms a distinctive subgroup of rare and highly aggressive cutaneous malignancies with poor prognosis. Approximately 50% of cutaneous ASs are found on the scalp and face, commonly in elderly men.1 Effective treatment options are limited for patients with metastatic disease. The current standard treatment is doxorubicin‐based chemotherapy. Due to a lack of clinical studies, there is no recommended second‐line regimen. We report a 59‐year‐old man who presented with bruise‐like macules over the forehead and scalp, and multiple bluish to red nodules on both temple regions (Fig. 1a,b). Pathology revealed a predominantly poorly differentiated angiosarcoma (Fig. 2). At that time, tumour staging showed no metastases. However, the primary tumour was already too widespread and multifocally distributed to allow complete surgical excision. Thus, a first‐line treatment was started with 13 cycles of pegylated liposomal doxorubicin (PLD) at a dose of 25 mg m−2 intravenously at 14‐day intervals combined with radiotherapy of the total scalp commencing with intensify‐modulated radiotherapy with photon beams (6 MV) at daily fractions of 2·5 Gy to a cumulative dose of 42·5 Gy, followed by lateral ‘opposed’ electron beams to a total dose of 60 Gy. Finally, the larger lesions received a boost by means of electron beams to a total cumulative dose of 70 Gy. Restaging revealed a complete remission of all tumours (Fig. 1c). Three weeks later, the patient developed cervical lymph node metastases of the left cheek, outside the irradiated region, and neck (Fig. 1d). After radiotherapy of the left preauricular region and lymph nodes on both sides of the neck (computed tomography‐planned, 3D conformal treatment planning with multiple photon beams, total dose of 50 Gy) followed by a boost to a cumulative dose of 70 Gy, restaging revealed a partial resolution of the tumours. Due to the residual tumour masses, we decided to start a second‐line chemotherapy using paclitaxel intravenously at a dose of 100 mg m−2 once weekly for 2 months, followed by 14‐day intervals for an additional 4 months up to a total of 15 cycles. A premedication including dexamethasone, cimetidine and ranitidine was given, and no obvious side‐effects were observed. Restaging demonstrated a complete remission of the primary tumour recurrence and of all lymph node metastases, which lasted for 11 months (Fig. 1e). Recently, the patient developed a locoregional relapse of the AS in the area of the left eyebrow, which was treated by surgical excision followed by radiotherapy. However, to date no distant metastases have been observed.