ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2020
RET gene rearrangements occur in a subset of pancreatic acinar cell carcinomas.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 39 citations in OpenAlex.
- Pancreatic Acinar Cell Carcinoma: A Rare Pancreatic Malignancy with Distinct Biology and Emerging Therapeutic Opportunities.Cancers · 2026Review
- Article
- Pediatric pancreatic acinar cell carcinoma with a non-canonical BRAF-KMT2C fusion and a classic SND1-BRAF fusion: a case report and literature review.BMC pediatrics · 2025Review
- Molecular and Clinical Features of Pancreatic Acinar Cell Carcinoma: A Single-Institution Case Series.Cancers · 2024Article
- Paediatric pancreatic acinar cell carcinoma with a novel SEC31A-BRAF fusion gene.Virchows Archiv : an international journal of pathology · 2024Article
- Fusion genes in pancreatic tumors.Trends in cancer · 2024Review
- Post-therapy emergence of an NBN reversion mutation in a patient with pancreatic acinar cell carcinoma.NPJ precision oncology · 2024Article
- A narrative review on rare types of pancreatic cancer: should they be treated as pancreatic ductal adenocarcinomas?Therapeutic advances in medical oncology · 2024Review
- Oncogenic Drivers and Therapeutic Vulnerabilities in KRAS Wild-Type Pancreatic Cancer.Clinical cancer research : an official journal of the American Association for Cancer Research · 2023Article
- Exceptional Responses to Selpercatinib inJCO precision oncology · 2023Article
- Review
- Two rare cancers of the exocrine pancreas: to treat or not to treat like ductal adenocarcinoma?Journal of cancer metastasis and treatment · 2023Article
- AGAP3: A novel BRAF fusion partner in pediatric pancreatic-type acinar cell carcinoma.Genes, chromosomes & cancer · 2022Article
- Pancreatoblastomas and mixed and pure acinar cell carcinomas share epigenetic signatures distinct from other neoplasms of the pancreas.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Article
- Molecular Characterization of KRAS Wild-type Tumors in Patients with Pancreatic Adenocarcinoma.Clinical cancer research : an official journal of the American Association for Cancer Research · 2022Article
- Imaging and Clinicopathological Features of Acinar Cell Carcinoma.Frontiers in oncology · 2022Review
- A Performance Comparison of Commonly Used Assays to Detect RET Fusions.Clinical cancer research : an official journal of the American Association for Cancer Research · 2021Article
- A Proposed Grading Scheme for Medullary Thyroid Carcinoma Based on Proliferative Activity (Ki-67 and Mitotic Count) and Coagulative Necrosis.The American journal of surgical pathology · 2020Article
- RAF1 rearrangements are common in pancreatic acinar cell carcinomas.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2020Article
- One size does not fit all for pancreatic cancers: A review on rare histologies and therapeutic approaches.World journal of gastrointestinal oncology · 2020Review
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Authors and funding
16 authors at 9 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Pancreatic acinar cell carcinoma is relatively rare (1 to 2% of pancreatic malignancies) but may be under-recognized. In contrast to pancreatic ductal adenocarcinoma, most acinar cell carcinomas lack mutations in KRAS, DPC, CDKN2A or TP53, but appear to have a high incidence of gene rearrangements, with up to 20% reported to be driven by BRAF fusions. With the development of a new class of RET-specific tyrosine kinase inhibitors, which appear to have particularly strong activity against RET gene rearranged tumours, there is now considerable interest in identifying RET gene rearrangements across a wide range of cancers. RET rearrangements have been reported to occur at a very low incidence (<1%) in all pancreatic carcinomas. We postulated that given its unique molecular profile, RET gene rearrangements may be common in acinar cell carcinomas. We performed fluorescent in-situ hybridization (FISH) studies on a cohort of 40 acinar cell spectrum tumours comprising 36 pure acinar cell carcinomas, three pancreatoblastomas and one mixed acinar-pancreatic neuroendocrine tumour. RET gene rearrangements were identified in 3 (7.5%) cases and BRAF gene rearrangements in 5 (12.5%). All gene rearranged tumours were pure acinar cell carcinomas. Our findings indicate that amongst all pancreatic carcinomas, acinar carcinomas are highly enriched for potentially actionable gene rearrangements in RET or BRAF. FISH testing is inexpensive and readily available in the routine clinical setting and may have a role in the assessment of all acinar cell carcinomas-at this stage to recruit patients for clinical trials of new targeted therapies, but perhaps in the near future as part of routine care.
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