DNA Mismatch Repair Abnormalities in Acinar Cell Carcinoma of the Pancreas

Liu, Weiguo; Shia, Jinru; Gönen, Mithat; Lowery, Maeve A.; O'Reilly, Eileen Mary; Klimstra, David S.

doi:10.1097/mpa.0000000000000190

Cited by 38 publications

(32 citation statements)

References 24 publications

(28 reference statements)

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“…Acinar cell carcinoma is an unusual tumor found in the context of Lynch syndrome with only four cases described in the literature (12, 13, 62). However, microsatellite instability has been evaluated in five different studies since Abraham and coworkers described three unstable ACCs in 2002 (13, 36, 46, 48, 62).…”

Section: Acinar Cell Carcinoma In Adultsmentioning

confidence: 99%

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

Rosa¹,

2015

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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.

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Section: Acinar Cell Carcinoma In Adultsmentioning

confidence: 99%

“…However, microsatellite instability has been evaluated in five different studies since Abraham and coworkers described three unstable ACCs in 2002 (13, 36, 46, 48, 62). Microsatellite instability has been investigated in a total of 159 ACCs and 13 of them (8.2%) were found to be unstable.…”

Section: Acinar Cell Carcinoma In Adultsmentioning

confidence: 99%

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

Rosa¹,

2015

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“…Since it is estimated that only 1%–2% of all pancreatic carcinoma are MMR deficient,148 the number of eligible patients with pancreatic cancer will be small. In contrast, MMR deficiency was reported to be more frequent in acinar cell carcinoma,149 and in intraductal papillary mucinous neoplasms (IPMN)-related tumours as compared with non-IPMN-related tumours 150…”

Section: Introductionmentioning

confidence: 99%

Stromal biology and therapy in pancreatic cancer: ready for clinical translation?

Neeße

Bauer

Öhlund

et al. 2018

Gut

269

222

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Pancreatic ductal adenocarcinoma (PDA) is notoriously aggressive and hard to treat. The tumour microenvironment (TME) in PDA is highly dynamic and has been found to promote tumour progression, metastasis niche formation and therapeutic resistance. Intensive research of recent years has revealed an incredible heterogeneity and complexity of the different components of the TME, including cancer-associated fibroblasts, immune cells, extracellular matrix components, tumour vessels and nerves. It has been hypothesised that paracrine interactions between neoplastic epithelial cells and TME compartments may result in either tumour-promoting or tumour-restraining consequences. A better preclinical understanding of such complex and dynamic network systems is required to develop more powerful treatment strategies for patients. Scientific activity and the number of compelling findings has virtually exploded during recent years. Here, we provide an update of the most recent findings in this area and discuss their translational and clinical implications for basic scientists and clinicians alike.

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“…Losses at 11p were also found in both pancreatoblastoma (80%) and ACC (50%) [23], [24], [78], [79]. Due to the rare cases of ACC in Lynch syndrome, mismatch repair abnormalities were studied in ACC and were found both in Lynch-associated and in 14% of sporadic cases [26], [25]. Finally, Carney syndrome has rarely been associated with ACC [27], and the PRKAR1A gene responsible for Carney syndrome is also mutated in some sporadic ACCs [80].…”

Section: Molecular Features Of Acinar Neoplasmsmentioning

confidence: 97%

“…Alpha-fetoprotein production can occur [22]. Hereditary cases are very unusual, although a number of cases have occurred in patients with familial adenomatous polyposis [23], [24], Lynch syndrome [25], [26], or Carney syndrome [27], providing some hints about the molecular underpinnings of acinar neoplasms (see below).…”

Section: Clinical and Pathological Features Of Acinar Cell Carcinomamentioning

confidence: 99%

Acinar neoplasms of the pancreas—A summary of 25 years of research

Klimstra

Adsay

2016

Seminars in Diagnostic Pathology

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DNA Mismatch Repair Abnormalities in Acinar Cell Carcinoma of the Pancreas

Abstract: 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.

Cited by 38 publications

References 24 publications

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

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

Stromal biology and therapy in pancreatic cancer: ready for clinical translation?

Acinar neoplasms of the pancreas—A summary of 25 years of research

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