Malignant hemangioendothelioma (MHE) of the thyroid still constitutes a relatively important part of our surgical material, with 23 MHE (2.0%) among 1153 primary thyroid tumors diagnosed between 1952 and 1987 (biopsy material of the Innsbruck Pathology Institute). 18 of these cases were investigated immunohistochemically; in 14 cases the data on clinical findings and follow-up were complete. Immunohistochemical results confirm the endothelial origin of this tumor and allow MHE to be definitively distinguished from anaplastic carcinomas. Follow-up and prognosis are determined by the aggressive nature of local tumor spread. 13 patients died after a median survival period of 2.4 months (R: 1.2-9.4). Only one patient has remained alive since over 4 1/2 years now. An improvement of the hitherto dismal prognosis is thought to depend on early tumor diagnosis and an ameliorated combined modality treatment.
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