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BackgroundAcinar cell carcinoma of the pancreas is a rare malignancy representing less than 1% of all pancreatic malignancies.MethodsWe report on a case series of 21 patients with acinar cell carcinoma of the pancreas treated at a high-volume quaternary center. A systematic review of the medical literature was performed that described typical therapeutic management approaches for acinar cell carcinoma of the pancreas and reported on disease control and survival rates. Data for the case series were obtained from a prospective database.ResultsIn our systematic review of 6 articles, study patients had a median age of 61 years, 66% were male, 52% had stage I/II disease, and 55% of lesions were located in the pancreatic head. The rates of median survival were approximately 47 months after resection with adjuvant therapy, 38 months for nonmetastatic, locally unresectable disease, and 17 months for metastatic disease treated with chemotherapy. Combination fluoropyrimidine-based chemotherapy regimens had better rates of disease control than other therapies. Our case series included 21 study patients, 14 of whom required resection and 7 who had metastatic disease. The rates of median survival were 40.2 + 31.9 months in those who underwent surgery and were treated with adjuvant therapy and 13.8 + 11.3 months for patients with metastatic disease.ConclusionsMultidisciplinary treatment for acinar cell carcinoma of the pancreas should be considered due to the rarity of the disease and its lack of high-level therapeutic data. Progress in the molecular analysis of this tumor may improve outcomes through the use of personalized therapy based on underlying tumor mutations.

Background: Patients with borderline resectable pancreatic cancer are at high risk of having positive surgical margins due to involvement of the tumor with adjacent vasculature. This article reviews the management of this subset of pancreatic cancer patients. Methods: The authors review the current definitions of borderline resectable pancreatic cancer and how it is diagnosed and staged. The history, current approaches, and future directions in neoadjuvant therapy for borderline resectable pancreatic cancer are also reviewed with emphasis on various chemotherapy regimens that have been used. The application of intensity-modulated radiation therapy and image-guided radiation therapy that accounts for respiratory motion to targeting the gross tumor volume in the pancreas are discussed, and the promise of integrating targeted therapies in neoadjuvant treatment programs is highlighted. Results: The use of neoadjuvant treatment programs that employ gemcitabine-based chemotherapy regimens followed by chemoradiation increases the likelihood of subsequent margin-negative resection in borderline resectable pancreatic cancer. Conclusions: There has been progress in the imaging, staging, surgical technique, and the use of chemotherapy and chemoradiotherapy in the management of borderline resectable pancreatic cancer. Patients can benefit from multidisciplinary management at high-volume pancreatic cancer treatment centers. Patients can benefit from the advances made in the assessment and treatment of borderline resectable pancreatic cancer.