27066 Angiosarcoma of the scalp and paraneoplastic encephalopathy: Multidisciplinary approach to diagnosis and treatment

Abstract

Cutaneous angiosarcomas of the scalp are rare tumors of the skin and soft tissue with an insidious onset and aggressive course. Paraneoplastic syndromes have been reported for skin cancers including melanoma and Merkel cell carcinoma. Here, we describe a case of a 75-year-old man with presumed paraneoplastic neurological syndrome in the context of angiosarcoma of the scalp. The patient initially presented with persistent frontal headaches which rapidly progressed to acute confusion, agitation, and word finding difficulties. CSF studies were negative for infection, and EEG demonstrated nonspecific slow waves. A brief course of high-dose steroids successfully treated his neurological symptoms; however his symptoms resurfaced with each taper. Upon hospitalization during which he was evaluated by a multidisciplinary team including neurology and dermatology, the etiology of his neurologic presentation remained elusive, until two cutaneous scalp lesions were identified and biopsied. Pathology confirmed the presence of angiosarcoma, and neuroimaging showed right parietal and vertex scalp soft tissue masses without bony or brain involvement. The patient responded to high-dose IV steroids and transitioned to oral prednisone 60 mg daily. Medical oncology was able to initiate chemotherapy successfully, but the patient eventually developed additional oncologic concerns. To our knowledge, this is one of the first reported cases of a paraneoplastic neurological syndrome occurring in close temporal association with cutaneous angiosarcoma of the scalp. Paraneoplastic syndromes may be considered in patients with various dermatological malignancies in general and in angiosarcoma. A multidisciplinary approach is key for these complex patient presentations.