Thyroid Angiosarcoma-Rare Case or Hard to Find

Thyroid Angiosarcoma is a rare malignant condition of the thyroid gland with higher incidence reported in mountainous Alpine regions (Switzerland, Austria and northern Italy), featured with poor prognosis.

A 76 years old male patient presented in our hospital complaining about difficulty in breathing, altered voice (hoarseness), fatigue and dysphagia. Careful observation of the frontal neck region revealed hemorrhaging petechiae and purpura, while palpation indicated a large nodule movable with deglutition, presumably derived by the thyroid gland. Serum biochemical thyroid function tests were normal. Ultrasound of the thyroid gland showed enlargement of the right lobe with a nodular lesion. FNAC indicated a "suspicious for malignancy" lesion. After patient's consensus, total thyroidectomy took place accompanied by excision of infiltrated infrahyoid muscles. The cytopathologic results were positive for primary thyroid angiosarcoma. Patient's postoperative condition was regular, but he died of disease progression 6 months later.

Angiosarcomas are malignant neoplasms arising from endothelial cells of blood vessels. They are most commonly found in skin, soft tissue, breast, bone, liver and spleen. Angiosarcomas tend to be highly hemorrhaging and invasive. Thyroid gland is a rare location of development. Cytopathologically they are grossly characterized by freely anastomosing vascular channels lined by atypical endothelial cells unusually enlarged, often multinucleated with many nucleoli and vacuoles into the cytoplasm containing fragments of erythrocytes. The immunohistological identity of angiosarcomas are endothelial line markers (CD31, CD34 and vimentin). Surgical excision when feasible is the first line treatment while adjuvant radio-and/or chemo-therapy are ambiguous. Infiltration of surrounding tissues and distant metastasis (lymph nodes and lungs) are negative prognostic factors.