Background: We describe a case of epithelioid angiosarcoma arising in the vagus nerve schwannoma of a 58 y old man.
Case presentation: Angiosarcoma rarely arises in a schwannoma, and epithelioid angiosarcoma arising in a vagus nerve schwannoma is exceedingly rare. We reported such a case in a 58 y old man who did not have neurofibromatosis. A mass on the right neck along the vagus nerve was noted to be present for six months and then was totally resected. After surgical resection, the neck location swelled/enlarged gradually over 6 months. Enhanced Magnetic Resonance Imaging (MRI) revealed an extremely invasive tumour with multiple enlarged lymph nodes of the bilateral neck that were diagnosed as a neoplasm recurrence with lymphatic metastasis and lymphovascular invasion. The reported cases and this case suggested a poor prognosis, including a high rate of recurrence, metastasis and death. Preoperative diagnosis, prognostic factors, and proposed histogenesis need to be discussed further.
Conclusions: We discuss the proposed histogenesis, clinical-pathological course, prognostic factors and literature on epithelioid angiosarcoma arising in vagus nerve schwannoma.