Angiosarcoma of the larynx. Case report and review of the literature.

Angiosarcoma of the larynx is a rare malignant tumour of vascular origin, accounting for less than 1% of all malignant tumours of the larynx. Angiosarcoma involves, in particular, the head and neck in areas such as the scalp and face. The causes are unknown, even if, in some cases, it is believed to be radiation-induced. The case is described of a patient with hypopharyngolaryngeal angiosarcoma, which became manifest with dysphagia, dysphonia and a palpable right latero-cervical mass about 7 cm in length. The patient underwent total pharyngolaryngectomy, right hemithyroidectomy, and bilateral neck dissection. Histological examination of the surgical specimen revealed a large haemorrhagic lesion involving the right pyriform sinus and homolateral hemilarynx. Right radical neck dissection revealed 9 metastatic lymph nodes, 1 of which with capsular invasion. Upon complete recovery the patient, underwent adjuvant post-operative radiotherapy. Six months later she is still alive with no clinical or radiological signs of disease. A careful review of the literature produced very few reports of such cases, only 6 of which in the last 30 years. Survival rate is very low, even if feasible average can be advanced, in view of the paucity of the case reports. Histological diagnosis is not always straightforward, as this neoplasm may be misdiagnosed as other vascular tumours (Kaposi's sarcoma, haemangiopericytoma), as non-neoplastic lesions (granulomas secondary to intubation) and as poorly differentiated squamous cell carcinoma. Immunohistochemical evaluation by means of markers, such as vimentin and factor VIII, offers a significant contribution to the diagnosis of angiosarcoma. The treatment of choice for laryngeal angiosarcoma is surgical excision, ample and radical, whenever possible, followed by adjuvant post-operative radiotherapy.