Laryngeal angiosarcoma: A clinicopathologic study of five cases with a review of the literature

Objective: Primary laryngeal angiosarcoma (LA) is rare without a reported s eries evaluating these tumors. Study Design/Methods: Five patients with LA were retrospectively retrieved from the Otorhinolaryngic Registry of the Ar med Forces Institute of Pathology. Results: Three men and 2 women, aged 29 to 71 years, presented with hoarseness (n = 4) and hemoptysis (n = 1), Two patients had previous neck radiation. The tumors involved the supraglottis (n = 4) with a mean size of 3.1 cm, Histologically, all tumors had anastomo sing vascular channels Lined by remarkably atypical endothelial cells protr uding into the lumen, frequent atypical mitotic figures, and hemorrhage, Ah cases tested (n = 4) demonstrated immunoreactivity with antibodies to Fact or VIII-RA and CD34, Ah patients had surgery followed by postoperative radi ation (n = 3 patients). Three patients died with disease (mean, 17 mo), whe reas one patient is alive with no evidence of disease at 18 years. Conclusi ons: LA is a rare tumor, frequently associated with previous radiation, usu ally involving the supraglottis with characteristic histomorphologic and im munophenotypic features. LA has a-poor prognosis, making appropriate separa tion from other conditions important.