Mixed pancreatic acinar cell-ductal adenocarcinoma: Complexities in diagnosis and treatment

Autor: Abdullah Nasser, Catherine L. Forse, Cynthia Walsh, Terence Moyana, Rakesh Goel
Jazyk: angličtina
Rok vydání: 2022
Předmět:
Zdroj: Current Problems in Cancer: Case Reports, Vol 5, Iss , Pp 100144- (2022)
Druh dokumentu: article
ISSN: 2666-6219
DOI: 10.1016/j.cpccr.2022.100144
Popis: Background: Acinar cell carcinoma of the pancreas (ACC) is a rare malignancy, accounting for approximately 1-2% of all pancreatic cancers. Up to a quarter of ACCs are estimated to contain a significant component of another histology such as neuroendocrine or ductal adenocarcinoma. Prognosis and management remain uncertain for these mixed tumors. Case: A 71-year-old was initially diagnosed with biopsy-proven metastatic pancreatic ACC and treated with FOLFOX. Subsequent biopsy of a progressing metastatic site revealed ductal adenocarcinoma, and liquid biopsy showed a PRKAR1A-RAF1 fusion. The patient had a partial response to FOLFOX and died nine months after initial diagnosis. Discussion: This case highlights a metastatic presentation of mixed pancreatic acinar cell-ductal adenocarcinoma, its reasonable response to treatment with FOLFOX, and overall survival which was driven by the more aggressive ductal component. We also report the presence of a RAF1 rearrangement, which may present a possible therapeutic target in these rare tumors.
Databáze: Directory of Open Access Journals