Clinicopathologic Study of 62 Acinar Cell Carcinomas of the Pancreas

• DOI: 10.1097/pas.0b013e318263209d
• Publication Date: 2012-10-01T02:54:47Z
• Authors: Stefano La Rosa; Volkan Adsay; Luca Albarello; Sofia Asioli; Selenia Casnedi; Francesca Franzi; Alessandro Marando; Kenji Notohara; Fausto Sessa; Alessandro Vanoli; Lizhi Zhang; Carlo Capella
• Keywords: Male; Acinar Cell; Predictive Value of Test; Kaplan-Meier Estimate; 0302 clinical medicine; Japan; morphology; 80 and over; acinar cell carcinoma; Multivariate Analysi; Aged, 80 and over; Tumor; Pancreatic Neoplasm; Middle Aged; Necrosi; Immunohistochemistry; Tumor Burden; 3. Good health; Europe; pancrea; Lymphatic Metastasis; Female; Anatomy; Human; United State; Adult; Prognosi; 2734; Risk Assessment; Immunophenotyping; Necrosis; 03 medical and health sciences; Biomarkers, Tumor; Humans; Neoplasm Invasiveness; Aged; Cell Proliferation; Neoplasm Staging; Neoplasm Invasivene; Carcinoma, Acinar Cell; Risk Factor; Carcinoma; Lymphatic Metastasi; acinar cell carcinoma; Pancreas; Prognostic markers; acinar cell carcinoma; morphology; pancreas; prognostic markers; Multivariate Analysis; Proportional Hazards Model; Surgery; prognostic marker; Biomarkers

Acinar cell carcinoma (ACC) of the pancreas is a very rare tumor that has various morphologic features, which may give rise to diagnostic difficulties. Because of its rarity, many clinicopathologic characteristics remain to be further elucidated, and prognostic factors are yet to be well established. With the aim of better characterizing this carcinoma and searching for prognostic indicators, we collected 62 ACCs and investigated the following parameters: site, size, local infiltration, node and distant metastases, architectural pattern, nuclear atypia, presence of necrosis, lymphovascular and perineural invasion, proliferation, BCL10, trypsin, carboxyl ester lipase, amylase, lipase, PDX1, cytokeratin 19 (CK19), CK7, p53, and β-catenin expression. Twelve cases showing >30% of endocrine cells were reclassified as mixed acinar-neuroendocrine carcinomas, whereas 1 tumor was reclassified as a mixed ductal-acinar carcinoma and was excluded from the statistical prognostic evaluations. BCL10 and trypsin were the most reliable immunohistochemical markers, whereas amylase and lipase were not. Surgery was statistically correlated with a better prognosis (P=0.0008). Among resected tumors there was no difference in survival between ACCs and mixed acinar-neuroendocrine carcinomas, and factors that significantly correlated with poor prognosis were size >6.5 cm (P=0.004), lymph node (P=0.0039) and distant (P=0.008) metastases, and UICC stage (P=0.009). Stage was the only independent prognostic factor at multivariable analysis, and the best prognostic discrimination was observed on grouping together stages I and II and grouping together stages III and IV, suggesting a simplification of the UICC staging for such cancers. In addition, vascular and perineural invasion and CK19 and p53 expression showed a trend for poor prognosis, not reaching statistical significance.