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; Ihsan E Demir; Fabio Casciani; Peter Kornprat; Doris Wagner; Christiane S Rösch; Giovanni Butturini; Alessandro Giardino; Peter E Goretzky; Martina Mogl; Tim Fahlbusch; Jörg Kaiser; Oliver Strobel; Anna Nießen; Andreas M Luu; Roberto Salvia; Manuel Maglione

doi:10.1016/j.hpb.2023.07.897

Treatment characteristics and outcomes of pure Acinar cell carcinoma of the pancreas - A multicentric European study on radically resected patients

HPB (Oxford). 2023 Nov;25(11):1411-1419. doi: 10.1016/j.hpb.2023.07.897. Epub 2023 Jul 27.

Authors

Ruben Bellotti¹, Salvatore Paiella², Florian Primavesi³, Carsten Jäger⁴, Ihsan E Demir⁴, Fabio Casciani², Peter Kornprat⁵, Doris Wagner⁵, Christiane S Rösch⁶, Giovanni Butturini⁷, Alessandro Giardino⁷, Peter E Goretzky⁸, Martina Mogl⁸, Tim Fahlbusch⁹, Jörg Kaiser¹⁰, Oliver Strobel¹¹, Anna Nießen¹⁰, Andreas M Luu¹², Roberto Salvia², Manuel Maglione¹³

Affiliations

¹ Department of Visceral, Transplant and Thoracic Surgery, Center of Operative Medicine, Medical University of Innsbruck, 6020 Innsbruck, Austria.
² General and Pancreatic Surgery Unit, Pancreas Institute, University of Verona, Verona, Italy.
³ Department of Visceral, Transplant and Thoracic Surgery, Center of Operative Medicine, Medical University of Innsbruck, 6020 Innsbruck, Austria; Department of General, Visceral and Vascular Surgery, Salzkammergut Hospital, 4840 Vöcklabruck, Austria.
⁴ Department of Surgery, Klinikum Rechts der Isar, School of Medicine, Technical University of Munich, Munich, Germany.
⁵ Department of General Surgery, Medical University of Graz, Graz, Austria.
⁶ Department of Surgery, Ordensklinikum Linz, Linz, Austria.
⁷ Department of Surgery, Pederzoli Hospital, Peschiera, Italy.
⁸ Department of Surgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin 13353, Germany.
⁹ St. Josef Hospital, Department of General and Visceral Surgery, Ruhr-University Bochum, Germany.
¹⁰ Department of General, Visceral and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany.
¹¹ Department of General, Visceral and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany; Division of Visceral Surgery, Department of General Surgery, Medical University of Vienna, Vienna, Austria.
¹² St. Josef Hospital, Department of General and Visceral Surgery, Ruhr-University Bochum, Germany; Klinikum für Allgemein, Viszeral- und Minimalinvasive Chirurgie, HELIOS Klinikum, Krefeld, Germany.
¹³ Department of Visceral, Transplant and Thoracic Surgery, Center of Operative Medicine, Medical University of Innsbruck, 6020 Innsbruck, Austria. Electronic address: manuel.maglione@i-med.ac.at.

PMID: 37563033
DOI: 10.1016/j.hpb.2023.07.897

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.