Retiform hemangioendothelioma (RH) is an extremely rare low-grade angiosarcoma mainly involving the skin and subcutaneous tissue. Clinically patients often present with an asymptomatic slow-growing solitary nodular or plaque-like lesion. RH is characterized by frequent local recurrences but a very low metastatic rate. Here we reported a case of RH in a 61-year-old Chinese woman who presented with a rapid growing cutaneous plaque-like lesion on her right scalp, followed by another lesion behind the right ear. The lesions were associated with paroxysmal sharp needle-stabbing like headache. She underwent wide excision and skin engraftment. Three months post surgery, she experienced tumor recurrence, and died 9 months after the initial diagnosis.
Keywords: Retiform hemangioendothelioma; headache; recurrence.