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WHO Classification of Tumours
Thyroid gland


A malignant tumor arising from the endothelial cells of the blood vessels. Microscopically, it is characterized by frequently open vascular anastomosing and branching channels. The malignant cells that line the vascular channels are spindle or epithelioid and often display hyperchromatic nuclei. Angiosarcomas most frequently occur in the skin and breast. Patients with long-standing lymphedema are at increased risk of developing angiosarcoma
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Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002


Angiosarcomas of thyroid were originally reported in Alpine countries of central Europe, and were linked to dietary iodine deficiency

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Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004

. This view has been challenged by cases occurring in flatland areas or seaside regions, suggesting that other as yet unknown factors may be involved in their etiology. Most patients die within less than six months after diagnosis, regardless of the treatment protocol
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Egloff B (1983)
The hemangioendothelioma of the thyroid.
Virchows Arch A Pathol Anat Histopathol 400: 119-42

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Ladurner D, Tötsch M, Luze T, Bangerl I, Sandbichler P, Schmid KW (1990)
[Malignant hemangioendothelioma of the thyroid gland. Pathology, clinical aspects and prognosis].
Wien Klin Wochenschr 102: 256-9

. Entirely intrathyroid tumours generally have a longer survival than those with extrathyroidal extension.