Efficacy of Chemotherapy in Patients with Unresectable or Metastatic Pancreatic Acinar Cell Carcinoma: Potentially Improved Efficacy with Oxaliplatin-Containing Regimen

Abstract

PurposePancreatic acinar cell carcinoma (ACC) is a rare cancer of the exocrine pancreas. Because of its rare incidence, the efficacy of chemotherapy in this patient population has been largely unknown. Therefore, we retrospectively analyzed the outcomes of patients with advanced pancreatic ACC who received chemotherapy.Materials and MethodsBetween January 1997 and March 2015, 15 patients with unresectable or metastatic pancreatic ACC who received systemic chemotherapy were identified in Asan Medical Center, Korea.ResultsThe median age was 58 years. Eleven and four patients had recurrent/metastatic and locally advanced unresectable disease. The median overall survival in all patients was 20.9 months (95% confidence interval [CI], 15.7 to 26.1). As first-line therapy, intravenous 5-fluorouracil were administered in four patients (27%), gemcitabine in five (33%), gemcitabine plus capecitabine in two (13%), oxaliplatin plus 5-fluorouracil/leucovorin (FOLFOX) in two (13%), and concurrent chemoradiotherapy followed by capecitabine maintenance therapy in two (13%). The objective response rate (ORR) to chemotherapy alone was 23% and the median progression-free survival (PFS) was 5.6 months (95% CI, 2.8 to 8.4). After progression, second-line chemotherapy was administered in eight patients, while four patients received FOLFOX and the other four patients received gemcitabine. The ORR was 38%, and patients administered FOLFOX had significantly better PFS than those administered gemcitabine (median, 6.5 months vs. 1.4 months; p=0.007). The ratio of time to tumor progression (TTP) during first-line chemotherapy to TTP at second-line chemotherapy was significantly higher in patients administered FOLFOX (4.07; range, 0.87 to 8.30) than in those administered gemcitabine (0.12; range, 0.08 to 0.25; p=0.029).ConclusionOur results suggest that oxaliplatin-containing regimens may have improved activity against pancreatic ACC.

Successful BRAF/MEK inhibition in a patient with BRAFV600E-mutated extrapancreatic acinar cell carcinoma

“…Small cohort studies and case reports list a number of regimens with an overall response rate of 23% and a median PFS of 5.6 mo (Al-Hader et al 2017;Yoo et al 2017). Chemotherapeutic substances include gemcitabine, capecitabine, 5-fluoruracil, irinotecan, cisplatin/oxaliplatin, and taxanes (Lowery et al 2011;Al-Hader et al 2017;Yoo et al 2017;Brunetti et al 2018) and also targeted therapies such as erlotinib or panitumumab in KRAS wild-type tumors (Morales et al 2013;Kruger et al 2016). Regarding predictors of treatment response, defects in DNA repair genes such as BRCA1, BRCA2, and PALB2 have been linked to prolonged survival following platinum-based regimens in some patients (Al-Hader et al 2017;Yoo et al 2017).…”

“…Chemotherapeutic substances include gemcitabine, capecitabine, 5-fluoruracil, irinotecan, cisplatin/oxaliplatin, and taxanes (Lowery et al 2011;Al-Hader et al 2017;Yoo et al 2017;Brunetti et al 2018) and also targeted therapies such as erlotinib or panitumumab in KRAS wild-type tumors (Morales et al 2013;Kruger et al 2016). Regarding predictors of treatment response, defects in DNA repair genes such as BRCA1, BRCA2, and PALB2 have been linked to prolonged survival following platinum-based regimens in some patients (Al-Hader et al 2017;Yoo et al 2017). Interestingly, the patient reported here had a germline heterozygous stop-gain mutation in PALB2, which has previously been reported in the context of familial pancreatic and breast cancer (Slater et al 2010).…”

Pancreatic acinar cell carcinoma—case report and literature review

“…There is no standard chemotherapy regime for unresectable pancreatic ACC cases. Yoo et al [ 18 ] confirmed that oxaliplatin-based chemotherapy had improved activity against pancreatic ACC as compared to gemcitabine. Hashimoto et al [ 9 ] suggested that modified FOLFIRINOX was safe and effective in the treatment of pancreatic ACC.…”

One size does not fit all for pancreatic cancers: A review on rare histologies and therapeutic approaches

“…To date, the contribution of adjuvant chemotherapy and radiotherapy has not been well investigated. However, considering the available data, resection and adjuvant chemotherapy are associated with the longest rate of mOS, whereas in some cases, chemoradiation has shown activity in both the neoadjuvant and locally advanced settings[ 5 , 8 , 9 , 25 - 28 ].…”