Angiosarcoma of the head and neck with intra-oral presentation. A clinico-pathological study of four cases

Abstract 

Angiosarcoma of the head and neck is a rare malignant tumour that most commonly involves the skin and subcutis, particularly of the scalp. It has been reported in intra-oral locations very rarely. This study reports on the clinico-pathological features of four such cases, in elderly patients and manifesting an aggressive course. All patients were admitted for rapidly enlarging masses involving the hard palate, alveolar crest and retromolar trigone and displaying bone destruction in three cases. Morphologically, three cases were composed by bundles of spindle cells admixed with variably sized abortive vascular channels which only showed slit-like spaces separating large clusters of epithelioid cells. In all instances, intra-cytoplasmic vacuoles containing intact or fragmented red blood cells were distinctly evident. All neoplasms showed consistent CD 31 immunoreactivity and variable degrees of CD 34 and Factor VIII related antigen positivity and were responsible for the death of the patients, for local progression or lung metastases, within 6 months from the original diagnosis. Due to their rarity, angiosarcomas with intra-oral presentation may be diagnosed with difficulty, especially on small incisional biopsies, and their differentiation from spindle cell carcinoma, malignant melanoma, anaplastic lymphoma, malignant myoepithelioma and other sarcomas that more commonly arise at this site should be based on accurate immunohistochemical characterisation.

Keywords:  Angiosarcoma, Head and neck tumours, Soft tissue tumours, Oral mucosa, Epithelioid angiosarcoma