Thyroid angiosarcoma is an aggressive and very rare type of thyroid malignancy originally diagnosed in iodine-deficient areas. The prognosis is poor and in most cases surgery proved to be inefficient. The fine needle aspiration biopsy (FNAB) and histology can rarely differentiate angiosarcoma from anaplastic carcinoma, and the final diagnosis is based on immunopositivity for vascular markers and absence of epithelial markers. We present here the case of primary thyroid angiosarcoma in a 52 years old male from a non-endemic area. He first presented with a rapidly enlarging cervical mass and the FNAB revealed anaplastic features. The patient received external beam radiotherapy with a total dose of 40 Gy. After an apparent tumoral volume reduction he underwent total thyroidectomy. Hematoxilin-eosin staining confirmed the undifferentiated malignant proliferation but the immunohistochemistry showed CD31, vimentin and FVIII positivity and thyroglobulin, calcitonin, TTF-1 and MNF116 negativity indicating an angiosarcoma. The patient started chemotherapy with pharmorubicin and cisplatin for tumor remnants yet with no effect on the tumor volume. Following three cycles of chemotherapy, 19 months after the diagnosis, the patient died due to respiratory failure. Whenever an apparently anaplastic thyroid carcinoma is associated with good outcomes in terms of survival and performance status we should look for a differential diagnosis.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology