Pancreatic Acinar Cell Carcinoma with Germline BRCA2 Mutation and Severe Pancreatic Panniculitis: A Case Report

Lena Dreikhausen; Nadine Schulte; Sebastian Belle; Philip Weidner; Johannes Moersdorf; Christoph Reissfelder; Matthias P Ebert; Tianzuo Zhan

doi:10.1159/000515267

Pancreatic Acinar Cell Carcinoma with Germline BRCA2 Mutation and Severe Pancreatic Panniculitis: A Case Report

Visc Med. 2021 Oct;37(5):447-450. doi: 10.1159/000515267. Epub 2021 Mar 29.

Authors

Lena Dreikhausen¹, Nadine Schulte¹, Sebastian Belle¹, Philip Weidner¹, Johannes Moersdorf², Christoph Reissfelder³, Matthias P Ebert¹, Tianzuo Zhan¹

Affiliations

¹ Department of Medicine II, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.
² Department of Dermatology, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.
³ Department of Surgery, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.

Abstract

Pancreatic acinar cell carcinoma (ACC) is a rare malignant disease that displays distinct differences to pancreatic ductal adenocarcinoma. Here, we report the case of a patient with ACC and underlying breast cancer susceptibility gene 2 (BRCA2) germline mutation that developed severe pancreatic panniculitis (PP) during the course of the disease. The patient received a multimodal therapy including surgery, systemic chemotherapy, and targeted therapy with the PARP inhibitor olaparib, resulting in an overall survival of 47 months. Findings from this case are compared to the current knowledge on management of ACC and paraneoplastic PP.

Keywords: Acinar cell carcinoma; BRCA2; FOLFIRINOX; Olaparib; Pancreatic panniculitis.

Publication types

Case Reports