Published online Apr 6, 2020. doi: 10.12998/wjcc.v8.i7.1241
Peer-review started: December 19, 2019
First decision: December 28, 2019
Revised: January 8, 2020
Accepted: March 11, 2020
Article in press: March 11, 2020
Published online: April 6, 2020
Processing time: 108 Days and 8.1 Hours
Pancreatic acinar cell carcinoma (PACC) is a rare type of malignant pancreatic cancer that represents approximately 1% of all pancreatic neoplasms. Due to its very low incidence, only a few retrospective studies are available. Although surgery is the first choice for treatment, most patients experience recurrence (mainly in the liver) and there are no clear recommendations for patients with advanced disease.
We report two patients with PACC treated with surgery who experienced tumour recurrence in the liver. Patient 1 carried a germline mutation in the APC gene. Both patients were treated with gemcitabine plus oxaliplatin and gemcitabine plus capecitabine as first- and second-line therapies, respectively. After a favourable response to chemotherapy, the patients underwent radiofrequency ablation of the remaining liver metastases. For patient 1, we documented a relapse in the liver after a disease-free period of 9 mo, and treatment with gemcitabine plus capecitabine was restarted. The patient achieved a complete response, and he remains alive without evidence of disease recurrence after six years. After radiofrequency ablation, patient 2 experienced disease-free survival for 21 mo, when peritoneal relapse was diagnosed and treated with chemotherapy. The patient achieved a stable disease state for nearly two years; nevertheless, further progressive disease was documented, and he died seven years after the first relapse.
PACC presents different biological behaviours than pancreatic adenocarcinoma. Multidisciplinary treatment involving local ablative therapies may be considered for PACC.
Core tip: Pancreatic acinar cell carcinoma (PACC) is a rare type of malignant pancreatic cancer. Surgery represents the first choice for treatment, but most patients experience relapse (mainly located in the liver), and there are no clear recommendations for the treatment of advanced disease. We report two cases of long-term PACC survivors treated with radiofrequency ablation in addition to chemotherapy for liver recurrence after surgery. This case report highlights the different biological behaviour of PACC compared to pancreatic adenocarcinoma and the importance of multidisciplinary treatment involving local ablative therapies, such as radiofrequency ablation, for recurrent PACC.