PURPOSE: A clinico-pathologic study of typical symptoms of intra- and extraoral angiosarcomas and clinical course under therapy is presented as well as an analysis of the immunohistochemical differential diagnosis of the tumour specific formed spaces. PATIENTS AND METHODS: Four male patients aged 63-78 years suffered from angiosarcomas of the maxillary sinus, the bucca (two patients) and the alveolar ridge of the lower jaw. HISTOPATHOLOGY: For comparative analysis paraffin embedded tissue of the initial biopsies was available. The slides were stained with standardized H&E, PAS and Gömörri. For standardized immunohistochemistry following primary antibodies were applied: monoclonal antibodies to pancytoceratin clones AE1/AE3, alpha-smooth-muscle-actin clone 1A4, CD31 clone JC/70A, factor-VIII-related antigen clone F/86, Fli-1 (polyclonal, Zymed, USA), tenascin-C: BC4 (Prof. L. Zardi), oncofetal glucosylated fibronectin clone FDC6 (ACCR), laminin-5: D4B5. Detection using AP-ChemMate and Autostainer (Dako, Denmark). RESULTS: While the benign appearance of the lesions resulted primarily in wrong diagnoses the histopathologic examination of the biopsies revealed the characteristic pattern of angiosarcomas. Wide surgical excision, radiotherapy and/or antiangiogenic chemotherapy could not prevent tumour progression and death within two and a half years after primary diagnosis. All angiosarcomas reacted partially positive for factor-VIII-related antigen and CD31. The tumour associated structural defect of vascular lamina with partial loss of pericytes/vascular smooth muscle cells was identified immunohistochemically by alpha-smooth-muscle-actin and for the first time by tenascin-C. CONCLUSIONS: (1.) The variable presentation and the benign appearance of oral and perioral angiosarcomas may often delay diagnosis. Oral and perioral angiosarcomas show poor prognosis despite of multimodal therapy. (2.) Cytoceratin and laminin-5-positivity as typical epithelial antigens don't exclude angiosarcoma. Factor-VIII-related antigen, CD31 as well as Fli-1 identify angiosarcoma. (3.) alpha-smooth-muscle-actin and the loss of the tenascin-C-matrix indicate immunohistochemically the characteristic sarcomatous defect of differentiation.
PURPOSE: A clinico-pathologic study of typical symptoms of intra- and extraoral angiosarcomas and clinical course under therapy is presented as well as an analysis of the immunohistochemical differential diagnosis of the tumour specific formed spaces. PATIENTS AND METHODS: Four male patients aged 63-78 years suffered from angiosarcomas of the maxillary sinus, the bucca (two patients) and the alveolar ridge of the lower jaw. HISTOPATHOLOGY: For comparative analysis paraffin embedded tissue of the initial biopsies was available. The slides were stained with standardized H&E, PAS and Gömörri. For standardized immunohistochemistry following primary antibodies were applied: monoclonal antibodies to pancytoceratin clones AE1/AE3, alpha-smooth-muscle-actin clone 1A4, CD31 clone JC/70A, factor-VIII-related antigen clone F/86, Fli-1 (polyclonal, Zymed, USA), tenascin-C: BC4 (Prof. L. Zardi), oncofetal glucosylated fibronectin clone FDC6 (ACCR), laminin-5: D4B5. Detection using AP-ChemMate and Autostainer (Dako, Denmark). RESULTS: While the benign appearance of the lesions resulted primarily in wrong diagnoses the histopathologic examination of the biopsies revealed the characteristic pattern of angiosarcomas. Wide surgical excision, radiotherapy and/or antiangiogenic chemotherapy could not prevent tumour progression and death within two and a half years after primary diagnosis. All angiosarcomas reacted partially positive for factor-VIII-related antigen and CD31. The tumour associated structural defect of vascular lamina with partial loss of pericytes/vascular smooth muscle cells was identified immunohistochemically by alpha-smooth-muscle-actin and for the first time by tenascin-C. CONCLUSIONS: (1.) The variable presentation and the benign appearance of oral and perioral angiosarcomas may often delay diagnosis. Oral and perioral angiosarcomas show poor prognosis despite of multimodal therapy. (2.) Cytoceratin and laminin-5-positivity as typical epithelial antigens don't exclude angiosarcoma. Factor-VIII-related antigen, CD31 as well as Fli-1 identify angiosarcoma. (3.) alpha-smooth-muscle-actin and the loss of the tenascin-C-matrix indicate immunohistochemically the characteristic sarcomatous defect of differentiation.