Endocrine Abstracts

Background: Primary thyroid angiosarcoma (PTA), previously considered a vascular type of anaplastic thyroid carcinoma (ATC), is a rare, aggressive malignant mesenchymal tumor, with a peak incidence in the seventh decade of life. PTA can present as an ATC, a more frequent type of thyroid malignancy. Differential diagnosis is of major importance, as the management and prognosis differ significantly.

Case report: A 72-year-old woman presented for a 2 month history of progressive obstructive respiratory symptoms. A large cervical mass identified on clinical exam was confirmed by the CT scan, showing a 93/90/76 mm inhomogeneous tumour on the topography of the left thyroid lobe, without metastasis. Thyroid function tests and calcitonin were normal. Surgery was imperative, given the significant respiratory symptoms due to adjacent organs’ tumor invasion assessed at surgical inspection. Total thyroidectomy and excision of the invaded infrahyoid muscles were performed, followed by a major improvement of the respiratory status. Histological analysis showed vasoformative high grade proliferation, hemorrhage, tumor necrosis, perienural and angioinvasion, and the residual thyroid gland had the aspect of multinodular goiter. Immunohistochemistry was negative for TTF-1, thyroglobulin and calcitonin and positive for the endothelial cell marker CD31, a hallmark for the diagnosis of angiosarcoma.

Conclusions: According to the literature data, PTA usually emerges in multinodular goitre. Although the large tumour mass invaded most of the thyroid tissue, our patient was euthyroid. The prognosis is poor, and it seems like angioinvasion may worsen the prognosis even more. In order to improve survival, early diagnosis, radical surgery and radiotherapy have proven their efficacy over time. Yet, due to its rarity, there is no gold standard treatment. Hence, patients may benefit from raising awareness and enrich clinical knowledge regarding this rare tumour.