Difficulty in diagnosing angiosarcoma of the scalp due to absence of purpura : A case report

Abstract

A 74-year-old woman presented with a multilocular tumorous lesion on the right side of scalp. Pathological examination of the biopsy specimen revealed dense infiltration of tumor cells with eosinophilic cytoplasm in the dermis. Definite histological diagnosis could not be obtained from hematoxylin and eosin staining or immunohistochemical analysis. At the time of total resection, two similar, small tumors were found in the left contralateral side of her scalp. Histopathological findings of both lesions revealed densely proliferative atypical endothelial cells and tumor cells that had formed an irregularly shaped luminal structure through the dermal layer. Immunohistochemical staining showed that the tumor cells were positive for CD31, D2-40 and vimentin. She was diagnosed with angiosarcoma. No metastatic lesion was found on positron emission tomography-computed tomography, and post-surgical treatment was not performed on the family's wishes. Ten months after the surgical excision, the tumor had metastasized to the lung and liver, and she was treated with two courses of weekly docetaxel therapy. The patient developed interstitial pneumonia, and, in spite of corticosteroid pulse therapy, she died without improvement. In this case, the presence of purpura, which is the usual characteristic of angiosarcoma, was not observed during the patient's clinical course. Therefore, we could not make a clinical diagnosis of angiosarcoma until complete resection. Taxane-based anticancer agents are frequently used as the first-line treatment for angiosarcoma of the scalp. Close attention needs to be paid to avoid the development of interstitial pneumonia.