Survival Outcome and Prognostic Factors for Pancreatic Acinar Cell Carcinoma: Retrospective Analysis from the German Cancer Registry Group
Less than 1% of all pancreatic malignancies are acinar cell carcinomas. Based on data from the German Cancer Registry Group, we performed a comparative analysis of characteristics and prognostic factors of pancreatic acinar cell carcinoma and the most common type of pancreatic cancer—pancreatic ductal adenocarcinoma. Compared to pancreatic ductal adenocarcinoma, patients with pancreatic acinar cell carcinoma were younger at the time of diagnosis and the percentage of males was higher. The prognosis of patients with pancreatic acinar carcinoma was better than that of patients with pancreatic ductal adenocarcinoma. Surgical resection was the strongest positive prognostic factor for pancreatic acinar cell carcinoma. The study shows that pancreatic acinar cell carcinoma has features distinct from pancreatic ductal adenocarcinoma. Radical resection should be advocated, whenever feasible.
不到 1% 的胰腺恶性肿瘤是腺泡细胞癌。基于德国癌症登记组的数据，我们对胰腺腺泡细胞癌和最常见的胰腺癌类型——胰腺导管腺癌的特征和预后因素进行了比较分析。与胰腺导管腺癌相比，胰腺腺泡细胞癌患者在诊断时更年轻，男性比例更高。胰腺腺泡癌患者的预后优于胰腺导管腺癌患者。手术切除是胰腺腺泡细胞癌最强的阳性预后因素。研究表明，胰腺腺泡细胞癌具有不同于胰腺导管腺癌的特征。
Background: Pancreatic acinar cell carcinoma (PACC) is a distinct type of pancreatic cancer with low prevalence. We aimed to analyze prognostic factors and survival outcome for PACC in comparison to pancreatic ductal adenocarcinoma (PDAC), based on data from the German Cancer Registry Group. Methods: Patients with PACC and PDAC were extracted from pooled data of the German clinical cancer registries (years 2000 to 2019). The distribution of demographic parameters, tumor stage and therapy modes were compared between PACC and PDAC. The Kaplan–Meier method and Cox regression analysis were used to delineate prognostic factors for PACC. Propensity score matching was used to compare survival between PACC and PDAC. Results: There were 233 (0.44%) patients with PACC out of 52,518 patients with pancreatic malignancy. Compared to PDAC, patients with PACC were younger (median age 66 versus 70, respectively, p < 0.001) and the percentage of males was higher (66.1% versus 53.3%, respectively, p < 0.001). More patients were resected with PACC than with PDAC (56.2% versus 38.9%, respectively, p < 0.001). The estimated overall median survival in PACC was 22 months (95% confidence interval 15 to 27), compared to 12 months (95% confidence interval 10 to 13) in the matched PDAC cohort (p < 0.001). Surgical resection was the strongest positive prognostic factor for PACC after adjusting for sex, age, and distant metastases (hazard ratio 0.34, 95% confidence interval 0.22 to 0.51, p < 0.001). There was no survival benefit for adjuvant therapy in PACC. Conclusions: PACC has overall better prognosis than PDAC. Surgical resection is the best therapeutic strategy for PACC and should be advocated even in advanced tumor stages.
胰腺腺泡细胞癌 (PACC) 是一种独特类型的胰腺癌，发病率较低。我们旨在根据德国癌症登记组的数据分析 PACC 与胰腺导管腺癌 (PDAC) 的预后因素和生存结果。方法：从德国临床癌症登记处（2000 年至 2019 年）的汇总数据中提取 PACC 和 PDAC 患者。比较 PACC 和 PDAC 的人口统计学参数分布、肿瘤分期和治疗模式。Kaplan-Meier 方法和 Cox 回归分析用于描述 PACC 的预后因素。倾向评分匹配用于比较 PACC 和 PDAC 之间的生存率。结果：在 52,518 名胰腺恶性肿瘤患者中，有 233 名 (0.44%) 患者患有 PACC。与 PDAC 相比，p < 0.001）并且男性比例更高（分别为 66.1% 和 53.3%，p < 0.001）。与 PDAC 相比，使用 PACC 切除的患者更多（分别为 56.2% 和 38.9%，p < 0.001）。PACC 中估计的总中位生存期为 22 个月（95% 置信区间 15 至 27），而匹配的 PDAC 队列为 12 个月（95% 置信区间 10 至 13）（p < 0.001）。在调整性别、年龄和远处转移后，手术切除是 PACC 最强的阳性预后因素（风险比 0.34，95% 置信区间 0.22 至 0.51，p< 0.001)。PACC 的辅助治疗没有生存获益。结论：PACC的总体预后优于PDAC。手术切除是 PACC 的最佳治疗策略，即使在晚期肿瘤阶段也应提倡。
It is important to bear in mind that PACC has features distinct from PDAC. Radical surgical resection should be advocated, including resection of metastases, whenever feasible. Though the role of systemic therapy is not well defined, it should be considered at least for patients with advanced disease.
重要的是要记住 PACC 具有与 PDAC 不同的特征。在可行的情况下，应提倡根治性手术切除，包括切除转移灶。尽管全身治疗的作用尚不明确，但至少应考虑对晚期疾病患者进行治疗。
关键词： 胰腺腺泡细胞癌,胰腺癌,德国癌症登记组,pancreatic acinar cell carcinoma,pancreatic cancer,German Cancer Registry Group