Background: Acinar cell carcinoma of the pancreas (ACC) accounts for approximately 1% of exocrine pancreatic tumors. Prognosis is poor, but recent studies suggest a better prognosis than ductal adenocarcinoma (DA). This study represents pooled data from multiple academic institutions to better understand the natural history and outcomes of patients with this rare form of pancreatic cancer.
Methods: Multi-institutional retrospective review of patients with ACC was conducted to evaluate the clinical presentation and outcomes of patients with ACC.
Results: Between the years 1988 and 2007, 16 patients were identified with acinar cell carcinoma (ACC) of the pancreas. Median age at presentation was 65 years. Patients commonly presented with abdominal (56%), back pain (44%) and weight loss (40%). Jaundice was not a typical presenting symptom. Fourteen patients underwent 15 operations: pancreaticoduodenectomy (8), distal pancreatectomy (4), and exploratory laparotomy (3). Three patients were found to be unresectable at initial operation, two with regionally advanced tumors and one with occult liver metastases. One patient with a regionally advanced tumor received neoadjuvant chemotherapy and was subsequently resected, and the other underwent chemoradiation but developed distant disease (supraclavicular node). Two patients were referred for surgery, but were managed non-operatively due to metastases. Mean tumor size was 5.5 ± 0.7 cm. AJCC tumor stages were stage I (1); stage II (9); stage III (3); and stage IV (3). In resected cases, 1 year survival was 100% and 5 year survival was 50%. Seven of 12 resected patients remain alive. One, the longest survivor to date (stage II), has survived 83 months. Two experienced hepatic metastases as a first sign of recurrence. One succumbed 13 months after distal pancreatectomy, and the other remains alive 76 months after pancreaticoduodenectomy. Patients with pre-operative metastases managed non-operatively were found to have a broad range of survival (1-63 months).
Conclusion: ACC of the pancreas is rare and has a presentation and outcome distinct from pancreatic ductal adenocarcinoma. Tumor size is larger at presentation, and prognosis in resected patients reflects other recent series demonstrating better survival. Patients with advanced disease managed non-operatively experience variable survival. A larger study is needed to examine treatment related outcomes and other predictors of survival in patients with ACC of the pancreas.