2022 Volume 83 Issue 9 Pages 1638-1643
A 51-year-old female presented to our hospital with complaints of appetite loss and abdominal pain. Computed tomography (CT) revealed a 16-cm cystic tumor with portal vein thrombosis. Additionally, endoscopic ultrasound-guided biopsy showed acinar cell carcinoma with positive Bcl-2 staining. We chose gemcitabine-S1 (GS) therapy for initial treatment. However, the patient's symptoms became worse during the first course. Thus, the patient underwent semi-emergent pancreatoduodenectomy and portal vein resection. The resected tumor was diagnosed as pancreatic acinar cell adenocarcinoma by histopathology. The postoperative course was uneventful without any adverse events. After discharge, recurrence of peritoneal dissemination was observed before adjuvant chemotherapy. Therefore, the patient was administered FOLFILINOX following four courses of GS therapy. However, the patient died after 14 months postoperatively. This case shows that in patients with pancreatic acinar cell carcinoma, chemotherapeutic response indicates prognosis.