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Angiosarcoma is a rare soft tissue highly malignant tumor of vascular origin, accounting for only 1% to 2% of these tumors. We present a rare case of De novo (unrelated to irradiation or pyothorax) angiosarcoma of the thoracic outlet with a 10-year disease free survival. Α 49-year-old male was admitted to our department due to a tumor of the thoracic outlet referred by the neurologists to whom he addressed complaining for vertigo and instability. After a thorough examination including chest X-ray, CT scan and MRI, a well circumscribed vascularized lesion of a diameter of 5cm was detected in the thoracic outlet. A head and neck angiography was performed along with a full staging in order to exclude metastatic disease. The patient was submitted to high axillary thoracotomy. The adjacent structures were dissected, small arterioles arising from the subclavian artery, neo-vessels were ligated or cauterized and the soft largely encapsulated tumor was excised. Gross observation of the resected specimen demonstrated a regular-shaped neoplasm. Histology revealed a well differentiated angiosarcoma comprised of multiple anastomosing blood vessels lined by endothelial cells showing malignant features but with little nuclear pleomorphism. The recovery was uneventful. The patient did not receive postoperative radiation or chemotherapy and 10 years postoperatively is free of disease. In conclusion, de novo primary pleural angiosarcoma are rare entities that should not be misdiagnosed. The immunohistochemical examination is the key for diagnosis and will offer the definite histotype. Although the prognosis is bad, early detection will give the patient the best chance for successful surgical treatment. Our patient being well and asymptomatic after 10 years follow-up represents a rare case and the first mentioned in the literature with a location of the lesion in the thoracic outlet.

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