Recent advances in imaging techniques such as dynamic intravenous contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) have enabled us to detect pancreatic cysts, some of which are potentially malignant. As the histopathological diagnosis cannot be confirmed preoperatively, enlarged pancreatic cysts are usually removed surgically. We report a rare case of pancreatic angiomatosis in a 21-year-old woman who presented with vague abdominal pain, a feeling of fullness, and nausea. Abdominal ultrasonography, contrast-enhanced CT, and MRI showed a huge mass in the right upper abdomen. We performed pylorus-preserving pancreato-duodenectomy, and the histopathological examination of the excised specimen revealed that the tumor was angiomatosis, probably originating from parapancreatic fibro-adipose tissue. She is doing well and has no evidence of recurrence 8 years after the operation. A complete surgical excision should be performed as curative treatment for pancreatic angiomatosis, which is an extremely rare disease when not associated with Von Hippel-Lindau syndrome.
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