Angiosarcoma ICDOcode91203

Primary angiosarcomas of the larynx are exceedingly rare, with only a few well-documented reports. Despite the fact that nearly 50% of all angiosarcomas occur in the skin and superficial soft tissues of the head and neck, angiosarco-ma accounts for less than 0.1 % of all head and neck malignancies. Laryngeal angiosarcoma is twice as frequent in men with a mean age at presentation in the 7th decade of life. Symptoms are non-specific; previous radiation exposure is frequently noted. The supraglottis is affected more frequently, specifically the epiglottis, where an increasing size is associated with a worse clinical outcome. Tumours demonstrate the typical histomorphologic features of angiosarco-ma in other soft tissue sites. Tumour cells are consistently positive with Factor VIII-related antigen, CD34, and CD31. Contact ulcer, haemangioma, acantho-lytic squamous cell carcinoma and mucosal malignant melanoma are the principle differential diagnostic considerations. Surgical excision is the treatment

Fig. 3.50 Laryngeal angiosarcoma. A Intermediate power demonstrating the anastomosing vascular channels lined by atypical endothelial cells. B A high power showing nuclear atypia of the hobnailed endothelial cells in a laryngeal angiosarcoma. C Factor VIII R-Ag reacts strongly and diffusely with the neoplastic endothe-lial cells.

Fig. 3.50 Laryngeal angiosarcoma. A Intermediate power demonstrating the anastomosing vascular channels lined by atypical endothelial cells. B A high power showing nuclear atypia of the hobnailed endothelial cells in a laryngeal angiosarcoma. C Factor VIII R-Ag reacts strongly and diffusely with the neoplastic endothe-lial cells.

of choice, with radiation therapy as necessary, yielding an overall patient survival of about 50% at 2 years. Large tumour size and previous radiation portends a worse prognosis {1556,1692, 2044,2055,2280,2591,2847}.

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