Eslinger Cody, Seddighzadeh Bobak, Yee Claire, Elsabbagh Zaid, Pai Rish, Hartley Chris, Starr Jason, Bekaii-Saab Tanios, Halfdanarson Thorvardur R, Sonbol Mohamad Bassam
Department of Hematology and Oncology, Mayo Clinic, Phoenix, AZ.
Department of Clinical Trials and Biostatistics, Mayo Clinic, Phoenix, AZ.
JCO Precis Oncol. 2025 Jan;9:e2400450. doi: 10.1200/PO-24-00450. Epub 2025 Jan 7.
Pancreatic acinar cell carcinoma (PACC) is a rare and aggressive form of pancreatic cancer that originates in the acinar cells of the exocrine pancreas. In this study, we aimed to investigate the clinical and molecular characteristics of patients with PACC at our institution.
This was a retrospective study of patients with PACC seen at Mayo Clinic between 2002 and 2023. Baseline patient characteristics, tumor pathology, treatment strategies used, and survival outcomes were analyzed. Kaplan-Meier curves were estimated using newsurv macros in SAS.
The study included a total of 65 patients with PACC. The median age at diagnosis was 66 years. Almost half of the patients (48%) presented with resectable/borderline-resectable disease (n = 28). Five-year overall survival (OS) for resectable/borderline-resectable, locally advanced/unresectable, and metastatic disease were 72.0%, 21.6%, and 20.9%, respectively. Somatic and germline next-generation sequencing identified numerous potentially actionable targets including homologous recombination (43% somatic, 33% germline), alterations (29% somatic), and mismatch repair (14% somatic).
Our findings underscore the heterogeneity and aggressive nature of PACC. Despite the improved prognosis for patients with resectable/borderline-resectable disease, OS remains poor, particularly for those with locally advanced or metastatic disease. The identification of actionable molecular targets in a significant proportion of patients highlights the potential for personalized therapeutic approaches. Future research should focus on tailored treatment strategies to exploit these molecular vulnerabilities, which may offer new options for improving outcomes in this rare malignancy.
胰腺腺泡细胞癌（PACC）是一种罕见且侵袭性强的胰腺癌，起源于外分泌胰腺的腺泡细胞。在本研究中，我们旨在调查我院PACC患者的临床和分子特征。
这是一项对2002年至2023年间在梅奥诊所就诊的PACC患者的回顾性研究。分析了患者的基线特征、肿瘤病理学、使用的治疗策略和生存结果。使用SAS中的newsurv宏估计Kaplan-Meier曲线。
该研究共纳入65例PACC患者。诊断时的中位年龄为66岁。几乎一半的患者（48%）表现为可切除/边界可切除疾病（n = 28）。可切除/边界可切除、局部晚期/不可切除和转移性疾病的5年总生存率分别为72.0%、21.6%和20.9%。体细胞和种系二代测序确定了许多潜在的可操作靶点，包括同源重组（体细胞43%，种系33%）、改变（体细胞29%）和错配修复（体细胞14%）。
我们的研究结果强调了PACC的异质性和侵袭性。尽管可切除/边界可切除疾病患者的预后有所改善，但总生存率仍然较差，特别是对于局部晚期或转移性疾病患者。在相当一部分患者中发现可操作的分子靶点凸显了个性化治疗方法的潜力。未来的研究应专注于定制治疗策略以利用这些分子弱点，这可能为改善这种罕见恶性肿瘤的治疗结果提供新的选择。
