Abstract
Background: Angiosarcoma of the pancreas is a very rare neoplasm accounting for 0.1% of pancreatic malignancies. This tumor is biologically very aggressive and frequently diagnosed at an unresectable stage. Case Presentation: Herein, we report a case of an incidentally discovered angiosarcoma that was removed with a robotic distal pancreatectomy and discuss the current literature on this rare disease. Conclusion: This is the fifth reported case of primary angiosarcoma of the pancreas and the first case to be identified incidentally. Unlike the other cases where survival was limited due to advanced disease, the patient presented here underwent surgical resection and remains disease free after 1 year of follow-up.