Acinar Cell Carcinoma of the Pancreas: Overview of Clinicopathologic Features and Insights into the Molecular Pathology

Abstract

Acinar cell carcinomas (ACCs) of the pancreas are rare pancreatic neoplasms accounting for about 1–2% of pancreatic tumors in adults and about 15% in pediatric subjects. They show different clinical symptoms at presentation, different morphological features, different outcomes, and different molecular alterations. This heterogeneous clinicopathological spectrum may give rise to difficulties in the clinical and pathological diagnosis with consequential therapeutic and prognostic implications. The molecular mechanisms involved in the onset and progression of ACCs are still not completely understood, although in recent years, several attempts have been made to clarify the molecular mechanisms involved in ACC biology. In this paper, we will review the main clinicopathological and molecular features of pancreatic ACCs of both adult and pediatric subjects to give the reader a comprehensive overview of this rare tumor type.

Insulinoma‐associated protein 1 expression in primary and metastatic neuroendocrine neoplasms of the gastrointestinal and pancreaticobiliary tracts

“…None of our acinar cell carcinoma cases was reactive for INSM1. However, one of the cases was reactive for CgA, in keeping with established literature . Interestingly, one of our six cases of poorly differentiated breast carcinoma was reactive for INSM1, similarly to the initial findings of Rosenbaum et al ., in which one of their four cases was positive .…”

Clinical outcomes of patients with resectable pancreatic acinar cell carcinoma

“…Distant metastasis, rather than local recurrence, is the most frequent pattern; effective adjuvant chemotherapy may enhance the clinical outcomes of patients with pancreatic ACC through the eradication of micrometastases after surgery. Further efforts to evaluate the molecular characteristics of pancreatic ACC may be useful when choosing relevant chemotherapeutic agents for adjuvant treatment . The recently noted frequent inactivation of DNA repair genes in pancreatic ACC may suggest the potential use of platinum‐based chemotherapy as adjuvant therapy …”

The pancreatic niche inhibits the effectiveness of sunitinib treatment of pancreatic cancer

“…Current therapies for unresectable tumors, which are the most common ones, include chemotherapy administration based on gemcitabine, folifirinox or nab-paclitaxel, but these are very inefficient and only minimally improve patient survival, such that new therapeutic strategies with improved efficiency are urgently needed. In contrast, other types of less-frequent pancreatic tumors, such as acinar cell carcinomas [3] and pancreatic neuroendocrine tumors [4], are associated with better prognosis and longer survival rates.…”