title: Epithelioid haemangiosarcoma of the thyroid gland. Report of six cases from a non-Alpine region.
abstract: OBJECTIVE
  To report a series of six cases of thyroid haemangiosarcoma (HAS) from a non-Alpine region.
  
  
  RESULTS
  The patients were four females and two males, aged 54-81 years (average 68 years). The tumours presented as large haemorrhagic masses (diameter 40-70 mm, average 56 mm) with extensive necrosis. Histologically, they were composed of polymorphous epithelioid cells with vesicular nuclei and abundant eosinophilic cytoplasm with occasional intracytoplasmic lumina. Mitotic activity was high. Tumor cells expressed vimentin (6/6), CD31 (6/6), FVIII (5/6), CD34 (2/6), and cytokeratins (5/6). One tumour (1/6) over-expressed p53 protein in more than 20% of cells. Ultrastructurally, Weibel-Palade bodies were present (4/6). Clinical follow-up of four patients (range 3-24 months, median 9 months) showed that two of them have died of the disease 0.5 and 3 months after diagnosis, one died of unrelated causes (with 24 months' uneventful follow-up) and one is alive 21 months after operation with no evidence of disease.
  
  
  CONCLUSIONS
  Although thyroid HAS is usually regarded as an extremely aggressive neoplasm with a dismal prognosis similar to anaplastic carcinoma, one of our cases suggests that HAS can behave in a less aggressive way. The morphological, immunohistochemical and ultrastructural findings support the hypothesis that thyroid HAS is a distinct entity, unrelated to other thyroid malignancies.
authors.affiliation: Department of Pathology, Charles University Medical Faculty Hospital, Hradec Králové, Czech Republic. ryskaale@fnhk.cz
authors.forename: A
authors.initials: A
authors.lastname: Ryska
authors.forename: M
authors.initials: M
authors.lastname: Ludvíková
authors.forename: P
authors.initials: P
authors.lastname: Szépe
authors.forename: A
authors.initials: A
authors.lastname: Böör
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D015622:Epithelioid Cells / Q000737:chemistry / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006394:Hemangiosarcoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008875:Middle Aged; D008938:Mitosis; D011933:Reagent Kits, Diagnostic; D013964:Thyroid Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D020302:Weibel-Palade Bodies / Q000648:ultrastructure
chemical_list: D014408:Biomarkers, Tumor; D011933:Reagent Kits, Diagnostic