TY - JOUR
T1 - Pancreatic neoplasms with acinar differentiation
T2 - A review of pathologic and molecular features
AU - Thompson, Elizabeth D.
AU - Wood, Laura D.
N1 - Funding Information:
This project has been funded by NIH/NCI P50 CA62924, NIH/NIDDK K08 DK107781, the Sol Goldman Pancreatic Cancer Research Center, the Buffone Family Gastrointestinal Cancer Research Fund, a Kaya Tuncer Career Development Award in Gastrointestinal Cancer Prevention, an AGA-Bernard Lee Schwartz Foundation Research Scholar Award in Pancreatic Cancer, a Sidney Kimmel Foundation for Cancer Research Kimmel Scholar Award, an AACR-Incyte Corporation Career Development Award for Pancreatic Cancer Research, an American Cancer Society Research Scholar Grant, the Emerson Collective Cancer Research Fund, the Rolfe Pancreatic Cancer Foundation, the Joseph C. Monastra Foundation, the Gerald O Mann Charitable Foundation (Harriet and Allan Wulfstat, Trustees), Susan Wojcicki, and Denis Troper.
Publisher Copyright:
© 2020 College of American Pathologists. All rights reserved.
PY - 2020/7
Y1 - 2020/7
N2 - Context.-Pancreatic acinar lesions encompass a broad spectrum of malignant tumors and benign reactive processes affecting both adults and children, with clinical, pathologic, and molecular features that are distinct from more common ductal neoplasms. Accurate morphologic diagnosis and molecular assessment of these uncommon neoplasms is critical for effective patient care. Objective.-To review the clinical, pathologic, and molecular features of pancreatic neoplasms with acinar differentiation, the most common of which is acinar cell carcinoma but which also includes mixed carcinomas with acinar components, cystic acinar lesions, and pancreatoblastoma. Data Sources.-We assessed the current primary literature, as well as recently updated diagnostic manuals. Conclusions.-Pancreatic acinar neoplasms are a morphologically and molecularly heterogeneous group of diseases that are characterized by acinar differentiation of at least a subset of the neoplastic cells, defined either morphologically (granular cytoplasm, single prominent nucleoli) or immunohistochemically. Squamoid nests are a key morphologic feature of pancreatoblastoma. Alterations in WNT signaling and chromosomal 11p loss are common molecular features of both acinar cell carcinoma and pancreatoblastoma. Targetable molecular alterations in acinar carcinoma include BRAF rearrangements and DNA repair defects, including mismatch repair deficiency and BRCA pathway defects. For practicing pathologists, morphologic recognition of such acinar neoplasms is critical, and in the future, molecular diagnostics to identify lesions susceptible to targeted therapy will likely form an important component of patient care.
AB - Context.-Pancreatic acinar lesions encompass a broad spectrum of malignant tumors and benign reactive processes affecting both adults and children, with clinical, pathologic, and molecular features that are distinct from more common ductal neoplasms. Accurate morphologic diagnosis and molecular assessment of these uncommon neoplasms is critical for effective patient care. Objective.-To review the clinical, pathologic, and molecular features of pancreatic neoplasms with acinar differentiation, the most common of which is acinar cell carcinoma but which also includes mixed carcinomas with acinar components, cystic acinar lesions, and pancreatoblastoma. Data Sources.-We assessed the current primary literature, as well as recently updated diagnostic manuals. Conclusions.-Pancreatic acinar neoplasms are a morphologically and molecularly heterogeneous group of diseases that are characterized by acinar differentiation of at least a subset of the neoplastic cells, defined either morphologically (granular cytoplasm, single prominent nucleoli) or immunohistochemically. Squamoid nests are a key morphologic feature of pancreatoblastoma. Alterations in WNT signaling and chromosomal 11p loss are common molecular features of both acinar cell carcinoma and pancreatoblastoma. Targetable molecular alterations in acinar carcinoma include BRAF rearrangements and DNA repair defects, including mismatch repair deficiency and BRCA pathway defects. For practicing pathologists, morphologic recognition of such acinar neoplasms is critical, and in the future, molecular diagnostics to identify lesions susceptible to targeted therapy will likely form an important component of patient care.
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U2 - 10.5858/arpa.2019-0472-RA
DO - 10.5858/arpa.2019-0472-RA
M3 - Article
C2 - 31869246
AN - SCOPUS:85088295780
SN - 0003-9985
VL - 144
SP - 808
EP - 815
JO - Archives of Pathology and Laboratory Medicine
JF - Archives of Pathology and Laboratory Medicine
IS - 7
ER -