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.
Prognosis of advanced or metastatic pancreatic adenocarcinoma is poor, with a median overall survival between 6.8 and 11 months for all histological types .
Pancreatic acinar cell carcinoma (ACC) is a rare entity. Few series have been published, mainly on patients treated by surgery [2,3]. Few data are available about chemosensitivity in patients with metastatic ACC. Evidence is poor, mainly based on case reports .
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. Physical examination revealed a mild hepatomegaly. The patient had a good performance status (ECOG 1). No weight loss was reported.
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. There was no evidence of nodes. A liver biopsy showed an ACC. The octreoscan and tumor marker CA 19.9 concentration were normal.
In May 2001, chemotherapy with gemcitabine 1,000 mg/m2 and oxaliplatin 130 mg/m2 biweekly was started. Patient experienced grade 1 urticaria at cycle 4 and grade 2 thrombopenia, justifying a 75% dose reduction at cycle 7. GEMOX was stopped after 9 cycles for grade 2 neuropathy.
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. CT showed no sign of relapse.
In July 2012, 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.
In the literature, data on ACC are scarce. The mean age at diagnostic is 60 years, with a male preponderance (65%) [3,5]. 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. This paraneoplastic syndrome is associated with cancer progression .
In three studies published, few patients had metastatic disease, and no data on chemotherapy were provided [2,3,5].
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 [4,8,9,10,11,12]. However, no complete response was observed. Combinations including LV5FU2 or oxaliplatin seem to be the most effective regimens.
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 .
Our patient carried a BRCA2 genetic mutation. BRCA2 mutations are known to be associated with pancreatic cancer, representing 2% of the total pancreatic cancer cases [14,15]. This mutation is also associated with ACC . 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, long survival can be expected.
Statement of Ethics
This case report has been written with the patient's approval.
The authors have no conflicts of interest to declare.