OBJECTIVES: 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?
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
- Adenocarcinoma, Mucinous/drug therapy
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Carcinoma, Acinar Cell/drug therapy
- Carcinoma, Adenosquamous/drug therapy
- Carcinoma, Pancreatic Ductal/drug therapy
- Cystadenocarcinoma, Mucinous/drug therapy
- Kaplan-Meier Estimate
- Middle Aged
- Pancreatic Neoplasms/drug therapy
- Retrospective Studies
- Treatment Outcome