ENDOVASCULAR PAPILLARY ANGIOENDOTHELIOMA-LIKE TUMOR-ASSOCIATED WITH LYMPHEDEMA

A case of endovascular papillary angioendothelioma-like tumour associa ted with lymphoedema is described, Microscopically the tumour was comp osed of anastomosing vascular channels, some of which contained papill ary projections, producing tuft-like or glomeruloid appearances, The d ermis also showed moderate lymphoedema and lymphocyic infiltrate, The tumour resembled endovascular papillary angioendothelioma but also had several features that differed from typical examples: occurrence in a n old patient and less prominent endothelial hobnail features and lymp hocytic 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 bou nd ulex europaeus agglutinin 1; 2 immature endothelial cells with roun d nuclei and vacuolated or pale cytoplasm, which were strongly positiv e 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 b y flow cytometry. The patient was well without disease twelve months a fter the excision, We postulate that this tumour was caused by circula tory disturbance, namely lymphoedema associated with syringomyelia and a Charcot's joint.