Abstract

INTRODUCTION: Acinar cell carcinoma is a rare pancreatic exocrine tumor that comprises approximately 1-2% of adult pancreatic tumors. We present a case of this rare tumor in a patient who presented with recurrent GI bleed. CASE DESCRIPTION/METHODS: A 48 year female with history of brain AV Malformaation, Chiari malformation s/p brain surgery and platelet storage pool disease presented to our hospital with worsening fatigue and shortness of breath, and a question of melena, and was found to be anemic at Hgb of 6.5. She underwent an upper endoscopy which revealed bright red blood in the second part of duodenum with questionable AV Malformations that were treated with Argon Plasma Cauterization. She received blood and platelet transfusions and was discharged with Hb of 12.7. Several days later she had an outpatient colonoscopy which was normal. One month after that, she was readmitted with increasing fatigue, dyspnea and periumbilical abdominal pain and was found to have Hgb 8.7. She also noted to have recurrent melenas. A repeat upper endoscopy was performed with the addition of a side viewing duodenoscope. This revealed an ulcerated mass involving the ampulla with mild oozing of blood. Hemostasis was achieved with epinephrine injection after biopsies were taken. Biopsies revealed an epithelial neoplasm, but a specific diagnosis was elusive. Subsequent PET scan showed focal uptake in the second part of the duodenum. She underwent pylorus preserving Whipple without any complications. The pathology of the surgical specimen revealed the mass to be an acinar cell carcinoma. The pArient had a stable postoperative follow up and improved clinically with repeat Hgb three week later of 11.3. DISCUSSION: Acinar cell tumor is a rare pancreatic exocrine tumor that usually affects males more than female. Mean age at presentation is usually 60. The presenting symptoms are non-specific and include abdominal pain, nausea, vomiting and weight loss. As biliary tract involvement is rare, jaundice is uncommon. Lipase hypersecretory syndrome from elevated lipase secretion causing Schmid triad (subcutaneous fat necrosis, polyarthritis, eosinophilia) is also rare (<10%). In our case, the presentation with GI bleed is exceedingly unusual. The treatment of local disease is surgical. No clear treatment guideline exists for patients in whom complete surgical resection for curative intent is not possible.

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