Diagnosis and treatment of pancreatic acinar cell carcinoma

Abstract

Objective To investigate the clinical characteristics and surgical outcomes of pancreatic acinar cell carcinoma (PACC). Methods The clinical and follow up data of PACC patients treated with surgery in Zhongshan Hospital of Fudan University between 1999 and 2012 were analyzed retrospectively. Results A total of 16 PACC were identified including 13 male and 3 female patients. The age of the patients ranged from 38 to 71 with an average of 57 years old. Six patients presented as abdominal pain, while low back pain in 3 patients, abdominal distention in 2 patients, emaciation in 2 patients, jaundice in 2 patients and melena in 1 patient. Elevated CA19-9 level was observed in 8 patients and 2 patients had elevated serum CEA. The tumors were located in the uncinate process in 1 patient, head in 9, body and tail in 6. The superior mesenteric vein was invaded in 2 cases and 1 patient had hepatic artery invasion. The tumor invaded both the celiac trunk and splenic artery in 1 patient. One patient had just undergone intra-operative needle aspiration biopsy due to unresectable tumor. All the other 15 patients underwent surgical excision with R0 resection. Among the 10 patients received pancreaticoduodenectomy, 2 had superior mesenteric vein resection and replacement and 1 had hepatic artery resection. Five patients underwent distal pancreatectomy without spleen preservation. The mean size of these tumors was 5.7 cm×4.6 cm, 12 cases had a surrounding envelope, while lymphatic metastasis was observed in 8 cases. The follow up data of 15 patients were collected and the median postoperative survival was 21 months, and the survival rate of 1, 3, 5 year was 71.4%, 28.6%, 7.1%. Conclusions Pre-operative diagnosis of PACC is extremely difficult due to lack of specific clinical features and lab tests. Surgery is the first line treatment for PACC, and the prognosis of PACC is better than that of pancreatic ductal adenocarcinoma. Key words: Pancreatic neoplasms; Acinar cell carcinoma; Surgery; Prognosis