Ikezawa Kenji, Urabe Makiko, Kai Yugo, Takada Ryoji, Akita Hirofumi, Nagata Shigenori, Ohkawa Kazuyoshi
Department of Hepatobiliary and Pancreatic Oncology, Osaka International Cancer Institute, Osaka, Japan.
Department of Gastroenterological Surgery, Osaka International Cancer Institute, Osaka, Japan.
Jpn J Clin Oncol. 2024 Mar 9;54(3):271-281. doi: 10.1093/jjco/hyad176.
Pancreatic acinar cell carcinoma is a rare form (0.2-4.3%) of pancreatic neoplasm with unique clinical and molecular characteristics, which largely differ from pancreatic ductal adenocarcinoma. Pancreatic acinar cell carcinoma occurs more frequently in males and can occur in children. Serum lipase is elevated in 24-58% of patients with pancreatic acinar cell carcinoma. Pancreatic acinar cell carcinomas tend to be large at diagnosis (median tumour size: ~5 cm) and are frequently located in the pancreas head. Radiologically, pancreatic acinar cell carcinoma generally exhibits a solid appearance; however, necrosis, cystic changes and intratumoral haemorrhage can occur in larger lesions. Immunostaining is essential for the definitive diagnosis of pancreatic acinar cell carcinoma. Compared with pancreatic ductal adenocarcinoma, pancreatic acinar cell carcinoma has a more favourable prognosis. Although radical surgery is recommended for patients with pancreatic acinar cell carcinoma who do not have distant metastases, the recurrence rate is high. The effectiveness of adjuvant therapy for pancreatic acinar cell carcinoma is unclear. The response to FOLFIRINOX is generally favourable, and some patients achieve a complete response. Pancreatic acinar cell carcinoma has a different genomic profile compared with pancreatic ductal adenocarcinoma. Although genomic analyses have shown that pancreatic acinar cell carcinoma rarely has KRAS, TP53 and CDKN2A mutations, it has a higher prevalence of homologous recombination-related genes, including BRCA1/2 and ATM, than pancreatic ductal adenocarcinoma, suggesting high sensitivity to platinum-containing regimens and PARP inhibitors. Targeted therapies for genomic alternations are beneficial. Therefore, genetic testing is important for patients with pancreatic acinar cell carcinoma to choose the optimal therapeutic strategy.
胰腺腺泡细胞癌是一种罕见的胰腺肿瘤形式（占比0.2 - 4.3%），具有独特的临床和分子特征，与胰腺导管腺癌有很大差异。胰腺腺泡细胞癌在男性中更为常见，也可发生于儿童。24 - 58%的胰腺腺泡细胞癌患者血清脂肪酶升高。胰腺腺泡细胞癌在诊断时往往体积较大（肿瘤中位数大小：约5厘米），且常位于胰头。在影像学上，胰腺腺泡细胞癌通常表现为实性外观；然而，较大的病灶可出现坏死、囊性变和瘤内出血。免疫组化对胰腺腺泡细胞癌的确诊至关重要。与胰腺导管腺癌相比，胰腺腺泡细胞癌的预后较好。虽然对于无远处转移的胰腺腺泡细胞癌患者建议进行根治性手术，但复发率较高。胰腺腺泡细胞癌辅助治疗的效果尚不清楚。对FOLFIRINOX方案的反应总体良好，部分患者可实现完全缓解。与胰腺导管腺癌相比，胰腺腺泡细胞癌具有不同的基因组特征。虽然基因组分析表明胰腺腺泡细胞癌很少有KRAS、TP53和CDKN2A突变，但与胰腺导管腺癌相比，其同源重组相关基因（包括BRCA1/2和ATM）的发生率更高，提示对含铂方案和PARP抑制剂高度敏感。针对基因组改变的靶向治疗是有益的。因此，基因检测对于胰腺腺泡细胞癌患者选择最佳治疗策略很重要。
