Curative remnant total pancreatectomy for recurrent pancreatic acinar cell carcinoma: A case report

Introduction and importance: Acinar cell carcinoma (ACC) of the pancreas is a rare pancreatic cancer subtype (incidence, 0.5-2%) with unclear epidemiology and prognosis. Sometimes, repeat resection including total pancreatectomy is required for recurrence. We report a case of ACC recurring in the remnant pancreatic head after distal pancreatectomy that was successfully cured by remnant pancreatic resection following combination therapy with nab-paclitaxel (nab-PTX) and gemcitabine (GEM). Case presentation: A 64-year-old woman was referred for pancreatic tumour treatment. CT revealed a 46-mm tumour in the pancreatic body, and endoscopic ultrasonography-guided fine needle aspiration (EUS-FNA) indicated ACC. Distal pancreatectomy was performed, and the final diagnosis was ACC. CT 18 months later showed a 34-mm tumour in the remnant pancreatic head revealed as ACC by EUS-FNA. Portal vein invasion was apparent, so neoadjuvant chemotherapy with nab-PTX and GEM was administered, and remnant pancreatic resection (total pancreatectomy) was performed. No recurrence or distant metastasis was present more than 6 months later. Clinical discussion: Mean survival time for ACC is 18-47 months, and prognosis is good compared with pancreatic ductal adenocarcinoma (PDAC). ACC tends to extend and grow along the main pancreatic duct, which increases the recurrence rate to 50-60%. EUS and EUS-FNA were useful for evaluating tumour extension and diagnosis. Repeat pancreatic resection that included total pancreatectomy was feasible and could be performed safely. Conclusion: ACC has a better prognosis than PDAC but with a higher recurrence rate. Aggressive surgical resection that included remnant total pancreatotomy with chemotherapy was useful in treating ACC.