Brunetti Oronzo, Aprile Giuseppe, Marchetti Paolo, Vasile Enrico, Casadei Gardini Andrea, Scartozzi Mario, Barni Sandro, Delfanti Sara, De Vita Fernando, Di Costanzo Francesco, Milella Michele, Cella Chiara Alessandra, Berardi Rossana, Cataldo Ivana, Scarpa Aldo, Basile Debora, Mazzuca Federica, Graziano Giusi, Argentiero Antonella, Santini Daniele, Reni Michele, Cascinu Stefano, Silvestris Nicola
Medical Oncology Unit, Sant'Andrea Hospital, University of Rome La Sapienza, Rome.
Medical Oncology Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa.
Pancreas. 2018 Jul;47(6):759-771. doi: 10.1097/MPA.0000000000001063.
Two issues were put forth by clinicians in the management of the advanced stages of rare variants of pancreatic ductal adenocarcinoma and other exocrine histotypes with peculiar clinical and pathological features: Do chemotherapy regimens recommended in pancreatic ductal adenocarcinoma patients have a clinical activity in rare pancreatic tumors? Or should other chemotherapy combinations be considered in this subset of patients?
We conducted a multicenter retrospective study that collected data from 2005 to 2016 at 14 Italian cancer centers with the aim to evaluate tumor response and time to progression for first- and second-line and overall survival.
Of approximately 4300 exocrine pancreatic cancer patients, 79 advanced cases affected by rare histological types were identified, with pancreatic acinar cell cancer (n = 23), pancreatic adenosquamous cancer (n = 16), and mucinous cystic neoplasm with an associated invasive mucinous cystadenocarcinoma (n = 15) most represented. Survival analyses for each subgroup in relation with the different chemotherapy regimens showed the lack of statistical significance correlations.
Because of the lack of clinical trials in patients affected by these rare pancreatic histotypes, only their molecular classification would help clinicians in future therapeutic choice.
临床医生在治疗具有特殊临床和病理特征的胰腺导管腺癌罕见变体及其他外分泌组织学类型的晚期阶段时，提出了两个问题：胰腺导管腺癌患者推荐的化疗方案在罕见胰腺肿瘤中是否具有临床活性？或者在这部分患者中是否应考虑其他化疗组合？
我们进行了一项多中心回顾性研究，收集了2005年至2016年期间意大利14个癌症中心的数据，旨在评估一线和二线治疗的肿瘤反应、进展时间及总生存期。
在约4300例胰腺外分泌癌患者中，确定了79例受罕见组织学类型影响的晚期病例，其中胰腺腺泡细胞癌（n = 23）、胰腺腺鳞癌（n = 16）和伴有浸润性黏液性囊腺癌的黏液性囊性肿瘤（n = 15）最为常见。对每个亚组与不同化疗方案相关的生存分析显示，缺乏统计学意义上的相关性。
由于这些罕见胰腺组织学类型的患者缺乏临床试验，只有对其进行分子分类才能帮助临床医生在未来做出治疗选择。
