prognosis of advanced or metastatic pancreatic adenocarcinoma is poor , with a median overall survival between 6.8 and 11 months for all histological types .
herein we report a case of a patient treated by the combination of gemcitabine plus oxaliplatin ( gemox regimen ) with a complete radiological response .
a 50-year - old male complained of epigastric and right hypochondriac region pain in april 2001 .
a ct scan showed multiple cystic tumors in the liver and spleen , a pseudocystic tumor on the pancreatic isthmus and an unlimited mass on the pancreatic corpus and tail with peripancreatic effusion .
the octreoscan and tumor marker ca 19.9 concentration were normal . in may 2001 , chemotherapy with gemcitabine 1,000
patient experienced grade 1 urticaria at cycle 4 and grade 2 thrombopenia , justifying a 75% dose reduction at cycle 7 .
evaluation after 4 cycles showed partial response with a 50% reduction of the liver and spleen tumors .
after 9 cycles , a complete tumor response was obtained . since 2002 , physical examination and biology have remained normal .
the initial biopsy was reanalyzed , and the initial diagnosis was confirmed . at the age of 61 years ,
a localized prostatic adenocarcinoma was diagnosed , treated by radical prostatectomy . in 2013 , a personal and family history of breast cancers ( sister at 34 years , mother at 60 years and the grandmother ) and a bladder cancer ( brother ) led to a genetic consultation with brca1 and brca2 genetic testing .
the patient as well as a cousin and his two sons were found to carry a brca2 mutation . in january 2015
, the patient had no clinical symptoms , a sequelary segmentary portal hypertension and well - epithelialized varicose vein on gastroscopy and he was relapse free .
the mean age at diagnostic is 60 years , with a male preponderance ( 65% ) .
the most common diagnosis is due to mass effect such as abdominal pain , jaundice or weight loss .
this tumor is often associated with pancreatic panniculitis ( weber - christian disease ) , perhaps due to high serum lipase concentrations .
data on chemosensitivity are only available from case reports . different regimens of chemotherapy ( gemcitabine , lv5fu2 , oxaliplatin , cisplatin , irinotecan , docetaxel and paclitaxel ) were tested alone or in combination . nevertheless , the combination of gemcitabine plus oxaliplatin was not investigated .
objective response rates were described with folfirinox , folfox , lv5fu2-gemcitabine , lv5fu2 , weekly paclitaxel and lv5fu2-cisplatin . however , no complete response was observed .
acc is associated with a better prognosis than ductal adenocarcinoma at all stages of disease . in metastatic disease , schmidt et al .   described a 5-year overall survival rate of 17.2% versus 2.8% for ductal carcinoma . in unresected patients , wisnoski et al .   reported an overall survival of 25 versus 3 months .
the longest survival observed in the literature was 11 years compared to 14 years in our patient .
brca2 mutations are known to be associated with pancreatic cancer , representing 2% of the total pancreatic cancer cases .
nowadays , there are ongoing trials testing parp inhibitors that show promising results with regard to platinum - based chemotherapy regimens .
we reported the first case of metastatic acc treated with the gemox regimen . despite the lack of study in these patients , a chemotherapy regimen with oxaliplatin seems to be the most effective combination with long survival . in these patients
 pancreatic acinar cell carcinoma ( acc ) is a rare entity . 
 herein we present the case of a 50-year - old male patient with an unlimited mass on the pancreatic corpus and tail with peripancreatic effusion and multiple metastases in the liver and spleen . 
 a liver biopsy showed a pancreatic acc . 
 the patient received 9 cycles of gemcitabine plus oxaliplatin ( gemox regimen ) , which had to be stopped because of a persistent grade 2 neuropathy . 
 a ct scan showed complete response after 14 years . at the age of 61 years 
 , a localized prostatic cancer was diagnosed , treated by prostatectomy . 
 the patient carried a brca2 mutation . 
 none of the precedent case reports describe a chemosensibility to the gemox regimen . in spite of 
 the lack of study in these patients , chemotherapy with oxaliplatin seems to be the most effective . 
 long survival can be expected . 
Introduction
Case Presentation
Discussion
Statement of Ethics
Disclosure Statement