Clinicopathological features and therapy of pancreatic acinar cell carcinoma: report of 17 cases and review of literature

Abstract

Objective To investigate the clinicopathological features and therapy of pancreatic acinar cell carcinoma (PACC) in order to improve the diagnosis and therapy of PACC.Methods The clinical data of 17 patients with pathologically confirmed PACC,who were admitted from November 2005 to March 2014 to Department of Pancreatic Surgery,Changhai Hospital,Second Military Medical University,were retrospectively reviewed,and the clinicopathological features and therapeutic methods were summarized and analyzed.Results The 17 cases included 10 men (58.8％) and 7 women (41.2％),ranging from 37 to 77 years old with an average of 59 years old.The chief complaints of 11 cases were abdominal pain or abdominal distension,3 cases reported jaundice,3 cases was detected by medical check-up.The preoperative serum test of CA19-9 in 4 cases,CEA in 1 case,AFP in 1 case was higher than the normal upper limit.Ultrasound showed solid or cystic hypoechoic mass with less uniform internal echo; CT and/or MR,MRCP showed relatively clear boundary tumor with lack of blood supply,with or without biliary or pancreatic duct dilation.The tumors were located in the head of the pancreas in 6 cases,in the neck in 2 cases,and in the body or tail in 9 cases.The diameter of tumor was 1.5-7.3 cm with an average of 3.7 cm.Thirteen (76.5％) cases were solid,4(23.5％) cases with cystic degeneration.Immunohistochemical staining showed α1-ACT and CAM5.2 were positive in all cases.Three cases underwent drug susceptibility test,the inhibition ratio of taxol and 5-fluorouracil (5-Fu) was more than 70％.Two (14.3％) cases had recurrence at the 10 th and 17 th month postoperatively,and 5 cases had liver and/or lymph node metastasis in the 3rd to 19th month postoperatively.Till the deadline of follow-up 3 cases died,and the survival time was 19,43 and 49 months,respectively,with a median survival of 37 months,the remaining 11 cases were alive,with an average of 31.5 months survival.Conclusions The pancreatic acinar cell carcinoma is a rare tumor of the pancreas,and is lack of specific clinical manifestation.The positive expression rate of α1-ACT is 100％,and resection is the treatment of choice for localized PACC,while taxol or 5-Fu is the main chemotherapy eomponent,and the overall survival is poor. Key words: Acinar cell carcinoma ; Pancreatic neoplasm ; Pathology, clinical ; Therapy