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. 2016 Dec;142(12):2585-2591.
doi: 10.1007/s00432-016-2264-7. Epub 2016 Sep 14.

Acinar cell carcinoma of the pancreas: a rare disease with different diagnostic and therapeutic implications than ductal adenocarcinoma

Stephan Kruger  1 , Michael Haas  1 , Philipp Johannes Burger  1 , Steffen Ormanns  2 , Dominik Paul Modest  1 , Christoph Benedikt Westphalen  1 , Axel Kleespies  3 , Martin Kurt Angele  3 , Werner Hartwig  3 , Christiane Josephine Bruns  4 , Thomas Kirchner  2 , Jens Werner  3 , Volker Heinemann  1 , Stefan Boeck  5
Affiliations

Acinar cell carcinoma of the pancreas: a rare disease with different diagnostic and therapeutic implications than ductal adenocarcinoma

Stephan Kruger et al. J Cancer Res Clin Oncol. 2016 Dec.

Abstract

Purpose: Acinar cell carcinoma (ACC) of the pancreas is a very rare cancer, constituting 1 % of all malignant non-endocrine pancreatic tumors. Only very limited data exist to guide treatment in patients with advanced ACC.

Methods: Between 2000 and 2015, 15 patients with ACC were diagnosed and/or treated at our high-volume comprehensive cancer center. Medical records and correlating serum levels of the potential serum tumor markers CA 19-9, CEA and lipase were analyzed retrospectively.

Results: A substantial antitumor activity was observed for treatment regimens containing 5-FU and oxaliplatin with partial responses or prolonged disease stabilizations (>12 months) observed in 6 out of 7 patients (86 %). Activity was also observed for single-agent 5-FU and its oral prodrugs. Serum lipase levels were elevated in 7 of 12 patients with advanced disease (58 %), whereas CEA and CA 19-9 seemed to be of minor importance for ACC (elevated pre-treatment levels in 4/12 and 3/12 cases, respectively). In selected patients, repeated serum lipase measurements were available and accurately predicted response to chemotherapy and relapse after surgery.

Conclusions: 5-FU- and oxaliplatin-containing regimens are active in advanced ACC. Lipase kinetics may be a useful novel tool to monitor the course of disease as well as treatment effects in ACC.

Keywords: Acinar cell carcinoma; Chemotherapy; Pancreas; Pancreatic cancer; Serum lipase; Tumor marker.

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Conflict of interest statement

Stefan Boeck has received honoraria for scientific presentations from Celgene and Roche, research funding from Celgene, Clovis Oncology and Roche, travel grants from Celgene and Roche, and acted as consultant for Celgene and Baxalta. Michael Haas has received honoraria for scientific presentations from Celgene, research funding (for his institution) from Boehringer Ingelheim and Roche, and travel grants from Boehringer Ingelheim and Celgene. Volker Heinemann has received honoraria for scientific presentations from Baxalta, Celgene and Roche, research funding from Celgene and Roche, and acted as consultant for Baxalta, Celgene and Roche. Thomas Kirchner has received honoraria for scientific presentations from Merck KGaA, and AstraZeneca, research funding from Merck and Roche, and acted as consultant for Amgen, AstraZeneca, Merck, MSD, Pfizer and Roche. All other authors declare no conflict of interest.

Figures

Fig. 1
Fig. 1
Serum lipase levels and correlation to course of disease for three selected patients with advanced pancreatic acinar cell carcinoma. Abbreviations: ACC acinar cell carcinoma, CT computed tomography, DOD dead of disease, FOLFOX folinic acid, fluorouracil and oxaliplatin, FOLFIRI folinic acid, fluorouracil and irinotecan, FOLFIRINOX folinic acid, fluorouracil, oxaliplatin and irinotecan, Gem gemcitabine, PD progressive disease, PR partial response, S-1 tegafur/gimeracil/oteracil, SD stable disease
See this image and copyright information in PMC

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