ReviewJournal of the National Comprehensive Cancer Network : JNCCN2021
Targeting the NTRK Fusion Gene in Pancreatic Acinar Cell Carcinoma: A Case Report and Review of the Literature.
Review in Journal of the National Comprehensive Cancer Network : JNCCN, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 49 citations in OpenAlex.
- Pancreatic Acinar Cell Carcinoma: A Rare Pancreatic Malignancy with Distinct Biology and Emerging Therapeutic Opportunities.Cancers · 2026Review
- Preoperative transcatheter arterial embolization enables safe resection of a giant hypervascular pancreatic acinar cell carcinoma: A case report.Radiology case reports · 2026Article
- Pancreatic ductal adenocarcinoma: integrating molecular insights for targeted interventions.Signal transduction and targeted therapy · 2026Review
- Genome-based classification of pancreatic acinar cell carcinoma reveals similarities to KRAS wild-type PDAC.Journal of translational medicine · 2025Article
- Emerging therapeutic advancements in pancreatic cancer: a contemporary review.Discover oncology · 2025Review
- Metastatic pancreatic cancer with activatingWorld journal of clinical cases · 2025Article
- Oligometastatic pancreatic cancer: current state of management and emerging therapies.The oncologist · 2025Review
- Pharmacological inhibition of hypoxia induced acidosis employing a CAIX inhibitor sensitizes gemcitabine resistant PDAC cells.Scientific reports · 2025Article
- Metastatic pancreatic acinar cell carcinoma withJournal of gastrointestinal oncology · 2025Article
- Pancreatic Acinar Cell Carcinoma: Demographics, Treatment, and Survival Outcomes, A Retrospective Population-Based Study.Journal of gastrointestinal cancer · 2025Article
- 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
- Target and Gene-Based Therapeutic Strategies against Pancreatic Cancer: Current and Future Prospects.Current gene therapy · 2025Review
- Molecular and Clinical Features of Pancreatic Acinar Cell Carcinoma: A Single-Institution Case Series.Cancers · 2024Article
- Complete pathological response to pembrolizumab in pretreated pancreatic acinar cell carcinoma.Journal of cancer research and clinical oncology · 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
- Comprehensive review of pancreatic acinar cell carcinoma: epidemiology, diagnosis, molecular features and treatment.Japanese journal of clinical oncology · 2024Review
- Roflumilast inhibits tumor growth and migration in STK11/LKB1 deficient pancreatic cancer.Cell death discovery · 2024Article
- Repotrectinib in a Patient WithJCO 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
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Authors and funding
17 authors at 4 institutions in 1 country.
Funding
Abstract
Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic exocrine malignancy. Compared with the more common pancreatic ductal adenocarcinoma (PDAC), PACC is more common in younger White men, has earlier stages and a lower mean age (56 vs 70 years) at the time of presentation, and has a better prognosis. In addition to differences in demographic, histologic, and clinical characteristics, PACC has a genomic profile distinct from PDAC, with only rare mutations in TP53, KRAS, and p16 that are commonly found in PDAC. This case report presents a man aged 81 years who presented with a pancreatic body mass with peripancreatic lymph node enlargement. Biopsy of the mass showed acinar cell carcinoma. The patient underwent upfront surgical resection, followed by one cycle of adjuvant gemcitabine, with stoppage of therapy due to poor tolerance. Lower-dose gemcitabine was reintroduced after disease progression 6 months later. Nab-paclitaxel was added to gemcitabine after 6 cycles because of a continued increase in the size of peripancreatic lymph nodes. Combination chemotherapy was stopped after 4 cycles because of further disease progression with new liver metastasis. Molecular testing showed the presence of an SEL1L-NTRK1 fusion. Targeted therapy was started with the oral neurotrophic tropomyosin receptor kinase (NTRK) inhibitor larotrectinib at a dosage of 100 mg twice daily. At the time of writing, the patient has been on therapy for 13 months with an exceptional radiographic response and has not experienced any grade 3 adverse effects. To our knowledge, this is the first clinical report of an NTRK gene fusion in a patient with PACC. This case study highlights the significance of tumor molecular profiling in patients with pancreatic tumors, especially rare histologies.
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