Sridharan Vishwajith, Mino-Kenudson Mari, Cleary James M, Rahma Osama E, Perez Kimberly, Clark Jeffrey W, Clancy Thomas E, Rubinson Douglas A, Goyal Lipika, Bazerbachi Fateh, Visrodia Kavel H, Qadan Motaz, Parikh Aparna, Ferrone Cristina R, Casey Brenna W, Fernandez-Del Castillo Carlos, Ryan David Patrick, Lillemoe Keith D, Warshaw Andrew L, Krishnan Kumar, Hernandez-Barco Yasmin G
Division of Gastroenterology, Massachusetts General Hospital, Harvard School of Medicine, Boston, MA, USA.
Division of Pathology, Massachusetts General Hospital, Harvard School of Medicine, Boston, MA, USA.
Pancreatology. 2021 May 15. doi: 10.1016/j.pan.2021.05.011.
Acinar cell carcinoma (ACC) is a very rare tumor of the exocrine pancreas, representing less than 1% of all pancreatic malignancies. The majority of data regarding ACC are limited to small case series.
This is a retrospective study conducted at a large healthcare system from 1996 to 2019. Patients with pathologically confirmed ACC were included, and demographic data, tumor characteristics, and treatment outcomes were abstracted by chart review. Survival curves were obtained by using the Kaplan-Meier method and compared using the log-rank test.
Sixty-six patients with ACC were identified. The median patient age at diagnosis was 64, and 42% presented with metastatic disease. The majority presented with abdominal pain or pancreatitis (69%), and laboratory parameters did not correlate with tumor size, metastatic disease, or survival. Several somatic abnormalities were noted in tumors (BRCA2, TP53, and mismatch-repair genes). In patients with localized disease that underwent resection, the median time to develop metastatic lesions was 13 months. The median overall survival (OS) was 24.7 months from diagnosis, with a survival difference based on metastatic disease at diagnosis (median 15 vs 38 mos). Surgery was associated with improved survival in non-metastatic cases (p = 0.006) but not metastatic cases (p = 0.22), and chemotherapy showed OS benefit in metastatic disease (p < 0.01). Patients with metastatic ACC treated after 2010 utilized more platinum-based agents, and there was a OS benefit to FOLFOX or FOLFIRINOX chemotherapy compared to gemcitabine or capecitabine-based regimens (p = 0.006).
Pancreatic ACC patients often present with advanced disease. Surgery was associated with survival benefit among patients presenting with localized disease. The use of FOLFOX or FOLFIRINOX chemotherapy regimens was associated with improved OS in metastatic patients. These data add to our knowledge in this rare malignancy, and improves understanding about the genomic underpinnings, prognosis and treatment for acinar cancers.
腺泡细胞癌（ACC）是一种非常罕见的胰腺外分泌肿瘤，占所有胰腺恶性肿瘤的比例不到1%。关于ACC的大多数数据仅限于小病例系列。
这是一项在一个大型医疗系统中于1996年至2019年进行的回顾性研究。纳入经病理证实为ACC的患者，通过查阅病历提取人口统计学数据、肿瘤特征和治疗结果。采用Kaplan-Meier方法获得生存曲线，并使用对数秩检验进行比较。
共识别出66例ACC患者。诊断时的中位年龄为64岁，42%的患者出现转移性疾病。大多数患者表现为腹痛或胰腺炎（69%），实验室参数与肿瘤大小、转移性疾病或生存率无关。在肿瘤中发现了几种体细胞异常（BRCA2、TP53和错配修复基因）。在接受手术切除的局限性疾病患者中，发生转移性病变的中位时间为13个月。从诊断开始的中位总生存期（OS）为24.7个月，根据诊断时的转移性疾病存在生存差异（中位15个月对38个月）。手术与非转移性病例的生存率提高相关（p = 0.006），但与转移性病例无关（p = 0.22）；化疗在转移性疾病中显示出OS获益（p < 0.01）。2010年后接受治疗的转移性ACC患者更多地使用铂类药物，与基于吉西他滨或卡培他滨的方案相比，FOLFOX或FOLFIRINOX化疗有OS获益（p = 0.006）。
胰腺ACC患者常表现为晚期疾病。手术与局限性疾病患者的生存获益相关。FOLFOX或FOLFIRINOX化疗方案的使用与转移性患者的OS改善相关。这些数据增加了我们对这种罕见恶性肿瘤的认识，并提高了对腺泡癌的基因组基础、预后和治疗的理解。
