The contemporary trend in worsening prognosis of pancreatic acinar cell carcinoma: A population-based study

Abstract

Background Primary acinar cell carcinoma (ACC) is a rare exocrine tumor of the pancreas with unclear clinical characteristics. Our goal was to determine the incidence and update the clinical characteristics and outcomes of ACC. Methods Through the Surveillance, Epidemiology, and End Results (SEER) database, we identified 252 patients with the latest diagnosis of ACC (2004–2016). The age-adjusted incidence (AAI) was calculated using the SEER*Stat Software version 8.3.6. The Kaplan–Meier method was used to draw survival curves and differences among them were compared by the log-rank test. Cox proportional hazards models were used to evaluate factors that had independent predictive effects on the overall survival. Results The AAI of pancreatic ACC was on the rise with the mean age at diagnosis of 63.79±14.79 years. Most patients (15.9%) had poorer differentiated tumors. The patients presented with distant stage were 54.4% compared with 53.1% between 1988 and 2003. The 1-, 2-, and 5-years survival rates for pancreatic ACC patients were 53.5%, 34.6%,17.5%, respectively (compared with 78.5%, 67.0%, and 42.8%, between 1988 and 2003). The multivariate COX analysis showed that the patient's age, surgery, chemotherapy, and summary stage, but not marital status were independent prognosis factors for ACC. Conclusions Pancreatic ACC is a highly malignant tumor with an increasing incidence in recent years. The rate of distant metastasis is increasing and the survival rate is worse than in the past, suggesting that it may require more aggressive treatment and follow-up. Surgery, radiotherapy, and chemotherapy are all effective treatments, but prospective studies are still needed to verify them.

Pancreatic acinar cell carcinoma: A comprehensive review

“…A recent study shows that ACC has a better prognosis than adenocarcinoma. They found that the prognosis was deteriorating with a decrease of 25% in a 5-year survival rate to previously reported rates[ 66 ]. However, data from a few studies published and anecdotal reports hint that some patients with ACCs have a greater survival rate than expected; unfortunately, no parameters are available to identify this group of patients[ 67 , 68 ].…”

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

“…Commonly, the mean survival time with ACC is 18–47 months, and the prognosis is thought to be better than that for PDAC [9] , [12] . However, some authors are noting that the prognosis is becoming worse nowadays [13] . There is no helpful specialized treatment strategy for ACC, and surgical resection is being recommended for resectable cases such as those with PDAC.…”

Survival Outcome and Prognostic Factors for Pancreatic Acinar Cell Carcinoma: Retrospective Analysis from the German Cancer Registry Group

“…Most of the large-scale studies on PACC are based on data from the American National Cancer Database (NCDB) as well as from the Surveillance, Epidemiology, and End Results (SEER) registry [ 3 , 5 , 8 , 18 , 19 , 20 ]. Some of these studies analyze overlapping cohorts, focusing on different aspects [ 5 , 8 , 18 , 19 , 20 ]. Table 4 gives an overview of publications with more than 50 patients included.…”