Treatment characteristics and outcomes of pure Acinar cell carcinoma of the pancreas – A multicentric European study on radically resected patients
Ruben Bellotti, Salvatore Paiella, Florian Primavesi, Carsten Jäger, İhsan Ekin Demir, Fabio Casciani, Peter Kornprat, Doris Wagner, Christiane Sophie Rösch, Giovanni Butturini, Alessandro Giardino, Peter E. Goretzky, Martina T. Mogl, Tim Fahlbusch, Jörg Kaiser, Oliver Strobel, Anna Nießen, Andreas Minh Luu, Roberto Salvia, Manuel Maglione
Abstract
BACKGROUND: Acinar cell carcinomas (ACC) belong to the exocrine pancreatic malignancies. Due to their rarity, there is no consensus regarding treatment strategies for resectable ACC. METHODS: This is a retrospective multicentric study of radically resected pure pancreatic ACC. Primary endpoints were overall survival (OS) and disease-free survival (DFS). Further endpoints were oncologic outcomes related to tumor stage and therapeutic protocols. RESULTS: 59 patients (44 men) with a median age of 64 years were included. The median tumor size was 45.0 mm. 61.0% were pT3 (n = 36), nodal positivity rate was 37.3% (n = 22), and synchronous distant metastases were present in 10.1% of the patients (n = 6). 5-Years OS was 60.9% and median DFS 30 months. 24 out of 31 recurred systemically (n = 18 only systemic, n = 6 local and systemic). Regarding TNM-staging, only the N2-stage negatively influenced OS and DFS (p = 0.004, p = 0.001). Adjuvant treatment protocols (performed in 62.7%) did neither improve OS (p = 0.542) nor DFS (p = 0.159). In 9 cases, radical resection was achieved following neoadjuvant therapy. DISCUSSION: Radical surgery is currently the mainstay for resectable ACC, even for limited metastatic disease. Novel (neo)adjuvant treatment strategies are needed, since current systemic therapies do not result in a clear survival benefit in the perioperative setting.