DNA mismatch repair abnormalities in Acinar cell carcinoma of the pancreas: Frequency and clinical significance Journal Article

Authors: Liu, W. G.; Shia, J. R.; Gonen, M.; Lowery, M. A.; O'Reilly, E. M.; Klimstra, D. S.
Article Title: DNA mismatch repair abnormalities in Acinar cell carcinoma of the pancreas: Frequency and clinical significance
Abstract: Objectives: Acinar cell carcinoma (ACC), including its mixed variants, is a rare pancreatic malignancy. Recent reports have documented its occurrence in Lynch syndrome. Our aim was to evaluate the frequency and clinicopathologic significance of DNA mismatch repair (MMR) deficiency in ACCs in general. Methods: Mismatch repair protein expression was evaluated by immunohistochemistry in a series of 36 ACC cases that were treated at our institution and had sufficient clinical information and pathologic material. Results: Loss of MMR protein was observed in 5 ACCs (5/36, 14%): 2 lost MLH1/PMS2, 2 lost MSH2/MSH6, and 1 lost MSH6 alone. The 1 MSH6-deficient case and 1 of the 2 MSH2/MSH6-deficient cases had a known history of Lynch syndrome, carrying a germline mutation in MSH6 and MSH2, respectively. None of the 5 tumors showed distinctive morphology. Two of the 5 patients died of disease 6 and 21 months after diagnosis. In contrast, in the MMR-normal group, only 1 of 30 patients died of disease (median follow-up, 32.5 months). Conclusions: Mismatch repair protein deficiency is not uncommon in ACCs, occurring in 14% of the cases in this series. The MMR-deficient ACCs did not show distinctive morphologic features and were clinically no less aggressive than MMR-normal ACCs.
Keywords: immunohistochemistry; pancreas cancer; neoplasms; syndrome; microsatellite instability; lynch syndrome; colon-cancer; nonpolyposis colorectal-cancer; chromosome 11p; insights; of-the-literature; microsatellite-instability; apc/beta-catenin pathway; lynch
Journal Title: Pancreas
Volume: 43
Issue: 8
ISSN: 0885-3177
Publisher: Lippincott Williams & Wilkins  
Date Published: 2014-11-01
Start Page: 1264
End Page: 1270
Language: English
ACCESSION: WOS:000344362600021
PUBMED: 25058881
DOI: 10.1097/MPA.0000000000000190
Notes: Article -- Source: Wos
Altmetric Score
MSK Authors
  1. Mithat Gonen
    721 Gonen
  2. David S Klimstra
    857 Klimstra
  3. Jinru Shia
    466 Shia
  4. Maeve Aine Lowery
    98 Lowery
  5. Eileen O'Reilly
    325 O'Reilly
  6. Weiguo Liu
    6 Liu