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.
Visc Med

中文翻译：

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胰腺腺泡细胞癌伴生殖系 BRCA2 突变和严重胰腺脂膜炎：病例报告

胰腺腺泡细胞癌（ACC）是一种罕见的恶性疾病，与胰腺导管腺癌有明显差异。在这里，我们报告了一例患有 ACC 和潜在乳腺癌易感基因 2 (BRCA2) 种系突变的患者，该患者在疾病过程中发展为严重的胰腺脂膜炎 (PP)。患者接受了包括手术、全身化疗和 PARP 抑制剂奥拉帕尼靶向治疗在内的多模式治疗，总生存期为 47 个月。将此病例的结果与当前关于 ACC 和副肿瘤性 PP 管理的知识进行了比较。
粘性医学