A puzzling scalp nodule: uncommon presentation of a rare disease

An elderly lady presented with a gradually progressive painless scalp swelling for the past 5 months. A hard, nontender nodule was noted over the mid-occipital area with a lobulated surface. Differentials were dermatofibroma, dermatofibrosarcoma protuberans, and calcified appendageal tumors. Biopsy showed a vasoformative tumor invading the dermis as nests and sheets of large pleomorphic cells with prominent nucleoli and increased mitotic activity, E-26 transformation-specific-related gene (ERG), and cytokeratin positivity. So, the case was diagnosed as angiosarcoma. She was referred to the Oncology Department for further management. Angiosarcoma of the head and neck area presents as single-to-multiple bluish red nodules or plaques that may ulcerate or bleed. Angiosarcoma of the scalp usually occurs in elderly men, which is very aggressive. Outcome can be improved with early diagnosis. But here, the patient was female without any history of bleeding or ulceration. Hard consistency was clinically mimicking a calcified or fibrous tumor. So, in all elderly patients with nodular lesions over the scalp or head and neck region, the possibility of angiosarcoma should be kept in mind.