Endovascular papillary angioendothelioma-like tumour associated with lymphoedema

A case of endovascular papillary angioendothelioma-like tumour associated with lymphoedema is described. Microscopically, the tumour was composed of anastomosing vascular channels, some of which contained papillary projections, producing tuft-like or glomeruloid appearances. The dermis also showed moderate lymphoedema and lymphocytic infiltrate. The tumour resembled endovascular papillary angioendothelioma but also had several features that differed from typical examples: occurrence in an old patient and less prominent endothelial hobnail features and lymphocytic infiltrate. Three types of proliferating cells were observed: 1 mature flattened endothelial cells, which were strongly positive for endothelial markers (factor VIII-related antigen, CD31, CD34) and bound ulex europaeus agglutinin 1;2 immature endothelial cells with round nuclei and vacuolated or pale cytoplasm, which were strongly positive for CD31 and muscle-specific actin (HHF35) and focally positive for other endothelial markers; and 3 stromal spindle cells in papillary or glomeruloid areas, which were positive for vimentin, HHF35, and alpha-smooth muscle actin but negative for desmin. The tumour was diploid by flow cytometry. The patient was well without disease twelve months after the excision. We postulate that this tumour was caused by circulatory disturbance, namely lymphoedema associated with syringomyelia and a Charcot's joint.