Exceptional Clinical Response to Alectinib in Pancreatic Acinar Cell Carcinoma With a Novel ALK-KANK4 Gene Fusion

摘要

INTRODUCTION: Pancreatic acinar cell carcinoma (PACC) is a rare cancer accounting for approximately 1% of exocrine pancreatic tumors. If compared with the most frequent pancreatic ductal adenocarcinoma (PDAC), patients affected by PACC are diagnosed at a significantly younger age, present with an earlier stage disease, and, thus, are more likely to undergo potentially curative resection. Even if presenting in advanced unresectable stage, patients affected by PACC maintain a better prognosis than those with PDAC with a 5-year survival rate of 22%. However, evidence regarding systemic treatments is limited in this rare subtype of pancreatic neoplasms, so the role of chemotherapeutic regiments for PACC remains poorly defined, and novel therapies are urgently needed