Herein we report a case of hemangioma with a rare localization in the major duodenal papilla presenting with a recurrent intraluminal bleeding in a 60-years old patient. Late onset, clinical and endoscopic signs suspicious for malignancy and inability to rule out cancer by preoperative biopsy defined an aggressive surgical approach. The patient underwent classical Whipple procedure after failing an attempt of endovascular embolization of the posterior pancreatoduodenal artery with unfavorable surgical outcome. Available preoperative diagnostic modalities and their accuracy levels are discussed.
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