The solid pseudopapillary neoplasm (SPN) of the pancreas is an uncommon, low-grade malignant tumour, mostly seen in young women. We report a rare case of a 44-year-old female who presented with spontaneous rupture and upper gastrointestinal bleeding. The emergency endoscopy revealed a 2 cm bleeding ulcer on the duodenal bulb. A computer tomography scan (CT scan) showed a 6.7 cm mass, with solid and cystic components arising in the head of the pancreas. After achieving haemostasis, she was discharged. Two months later, a new CT scan showed a persistent 6 cm mass in the head of the pancreas, now containing air and communicating with the duodenal lumen. The patient was successfully treated by pancreatoduodenectomy. Histopathological examination showed a T3N0M0 SPN with immunohistochemical expression of β-catenin, synaptophysin, vimentin and progesterone receptor, and negativity for chromogranin. The labelling index of Ki 67 was 2%. No recurrence was present after 2 years of follow-up.
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