Analysis and literature review of pediatric pancreatic acinar cell carcinoma: a report of 3 cases

Abstract

Objective To explore the clinical characteristics and managements of pancreatic acinar cell carcinoma (PACC) in children. Methods Three PACC cases were selected from our center while another 1 case was collected from the databases of Wanfang, CMCC, CNKI, PubMed, Medline, BIOSIS Previews, Embase and Cochrane Library before January 2018. Results Three children from our center were diagnosed with PACC. Another PACC child was selected from literature search. There were 3 boys and 1 girl with a median age of 7 years. The preoperative complaints included abdominal mass (n=4) and abdominal pain (n=2). All neoplasms were single. And the sites were pancreatic head (n=1) and pancreatic tail (n=2). The average size of neoplasm was 75 mm×59 mm×53 mm. Postoperative immunohistochemistry was positive for α-AT while α-ACT and CgA (-) were negative. One patient from our center refused postoperative chemotherapy and died of tumor recurrence at 12 months. One case became lost to follow-ups after 18 months and another one survived to date. The child from literature search died of tumor metastasis and cachexia at 13 months postoperatively. The mean survival was 13.5 months. Conclusions It is difficult to make a preoperative definite diagnosis of PACC in children. The prognosis is relatively poor for children with distinct protein phenotypes. Aggressive surgery and postoperative chemotherapy may prolong patient survival. Key words: Pancreatic neoplasm; Acinic cell carcinoma; Child