We report on a case of a retroperitoneal accessory spleen with vascular supply directly from the aorta. A 47-year old woman presented with a six-month history of epigastric pain, intermittent nausea and vomiting. The CT scan of the abdomen revealed the presence of a retroperitoneal tumor between the spleen, left kidney and pancreas. The MR-tomography confirmed the presence of a solid tumor, with vascular supply directly from the aorta. The exploratory laparotomy showed the presence of a well-shaped accessory spleen, which was confirmed by the histological examination of the specimen. The surgeon should be aware of the possible existence of accessory spleens for the differential diagnosis of retroperitoneal tumors.
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