Cytomorphologic, immunophenotypical and molecular features of pancreatic acinar cell carcinoma

Abstract

ObjectivesAs a rare tumor in pancreas, pancreatic acinar cell carcinoma (PACC) possesses a distinct molecular feature from pancreatic ductal carcinoma (PDAC). Though the diagnosis of PACC is often established based on cytology specimens, its cytologic diagnosis can be challenging. Furthermore, the correlation between PACC cytomorphology and its unique different molecular alterations have not been fully explored.MethodsCytology features were analyzed in 8 histologically proven PACC and cytohistological correlation was performed. Immunocytochemistry for trypsin, chymotrypsin, BCL10, synaptophysin, chromogranin A, INSM1, β‐catenin, and Ki‐67 was assessed. Comprehensive molecular profiling and additional targetable treatment biomarker assessment were also performed.ResultsThe cohort included 4 mixed acinar‐neuroendocrine carcinomas, 3 pure PACCs, and 1 mixed acinar‐ductal carcinoma. Immunophenotypical features are consistent with diagnoses of PACC or PACC with neuroendocrine features. Identified genetic alterations included somatic mutations of CTNNB1, TP53, MAP2K1, PTEN, RAC1, germline mutations of NBN and BRAC2, and gene fusion of CCDC6‐RET.ConclusionsThe current study is the first attempt to explore the correlation between the cytomorphology characteristics and molecular features of PACC and a few intriguing findings were observed. Further validation in larger cohorts is warranted.

Comprehensive review of pancreatic acinar cell carcinoma: epidemiology, diagnosis, molecular features and treatment

“…For the definitive diagnosis of PACC, immunostaining with antibodies to identify acinar cell differentiation (trypsin and Bcl-10) ( Fig. 1 ), in addition to the use of general neuroendocrine markers (synaptophysin and Chromogranin A) to exclude pNEN, is important ( 16 , 44 , 52 , 53 ). Scattered neuroendocrine cells positive for synaptophysin and/or Chromogranin A can be observed in PACC, and a small subset of pNEN shows focal acinar differentiation ( 2 , 54 ).…”