Acinar cell carcinoma: a report of 19 cases with a brief review of the literature

Abstract

Background: Acinar cell carcinoma (ACC) is a relatively rare pancreatic neoplasm with poorly defined prognosis. This study aimed to investigate this rare pancreatic neoplasm through comparing patients with ACC to pancreatic ductal cell adenocarcinoma (DCA).

Methods: Tianjin Medical University Cancer Institute and Hospital pathology database was reviewed from 1995 to 2015, and 19 patients with pathologically confirmed ACC were enrolled while 19 conventional DCA patients assigned randomly as control. Retrospective review and follow-up were performed for each patient. Regression methods were used to identify differences between ACC and DCA.

Results: In our study, most patients suffered from abdominal or back pain, and no lipase hypersecretion syndrome was observed. For ACC, resected cases had better survival than those without resection, and earlier staging was related to longer survival. Resection with postoperative adjuvant therapy had a better outcome than surgery alone. Twelve cases developed recurrence. Compared to DCA, ACC had earlier staging and better survival. The overall 1-, 2-, and 5-year survival rates for patients with ACC were 73.7, 26.3, and 5 %, respectively.

Conclusions: ACC carries a better prognosis than DCA and a similarly high recurrence rate, while surgical resection proved the best first-line approach for it. A well-planned neoadjuvant or adjuvant chemoradiotherapy indeed benefit the patients with ACC.

Keywords: Acinar cell carcinoma; Pancreas; Pancreatectomy; Pancreatic cancer; Resection; Surgery.

Fig. 1

Schematic of patient treatment. Among 19 patients, three of them received palliative therapy because of the advanced diseases. The remaining 16 patients underwent exploratory laparotomy. Two of them were found unresectable, and the rest 14 patients received radical resection with R0 margins, 6 pancreaticoduodenectomy, and 8 pancreatectomy

Fig. 2

The Kaplan-Meier actuarial survival curves for ACC patients. a Overall stage-specific survival were stage I 21.0 % (mean survival 45.75 months), stage II 52.6 % (mean survival 19.4 months), stage III 10.5 % (mean survival 12.0 months), and stage IV 15.8 % (mean survival 8.33 months). b The resected cases had a significantly better survival than those without resection (median survival 19 vs. 9 months, P < 0.0001). c Among resected cases, earlier T classification was associated with a longer survival time (P < 0.05). d Resection followed by postoperative adjuvant therapy had a better outcome than surgery alone (P = 0.006)

Fig. 3

The Kaplan-Meier actuarial survival curves for the entire cohort by tumor type. The overall 1-, 2-, and 5-year survival rates for patients with ACC were 73.7, 26.3, and 5 %, respectively (median 18 months), whereas the overall survival rates for patients with DCA were 26.3, 2, and 0 %, respectively (median, 4 months, P < 0001)