ArticleJournal of gastrointestinal oncology2017
Pancreatic non-functioning neuroendocrine tumor: a new entity genetically related to Lynch syndrome.
Article in Journal of gastrointestinal oncology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 23 citations in OpenAlex.
- Metastatic Unfunctional Pancreatic Neuroendocrine Tumor in Lynch Syndrome.Clinical case reports · 2026Article
- Pancreatic Neuroendocrine Tumor in Lynch Syndrome: Expanding the Tumor Spectrum of Mismatch Repair Deficiency.Cureus · 2026Article
- Multi-omic profiles of neuroendocrine neoplasms of the pancreas: an integrated landscape.Molecular cancer · 2026Review
- Article
- Germline Mutations in DNA Repair Genes in Patients with Pancreatic Neuroendocrine Neoplasms: Diagnostic and Therapeutic Implications.Current oncology (Toronto, Ont.) · 2025Article
- Neuroendocrine neoplasms as a lynch syndrome manifestation: a case report and comprehensive literature review.Frontiers in endocrinology · 2025Review
- Risk of Gastric and Small Intestinal Cancer in Patients With Lynch Syndrome: Data From a Large, Community-Based US Population.Clinical and translational gastroenterology · 2024Article
- De novo germline pathogenic variant in Lynch Syndrome: A rare event or the tip of the iceberg?Tumori · 2024Article
- Pathogenic Insights into DNA Mismatch Repair (MMR) Genes-Proteins and Microsatellite Instability: Focus on Adrenocortical Carcinoma and Beyond.Diagnostics (Basel, Switzerland) · 2023Review
- Overview of the 2022 WHO Classification of Neuroendocrine Neoplasms.Endocrine pathology · 2022Review
- Using Immunohistochemistry to Expand the Spectrum of Lynch Syndrome-Related Tumors.ACG case reports journal · 2021Article
- Molecular Pathology of Well-Differentiated Gastro-entero-pancreatic Neuroendocrine Tumors.Endocrine pathology · 2021Review
- Case Report: Grade 2 Metastatic Pancreatic Neuroendocrine Tumor With Progression of One Metastasis After Pregnancy to Grade 3 Large-Cell Neuroendocrine Carcinoma: One Case Cured by Resection With Genomic Characterization of the Two Components.Frontiers in oncology · 2021Article
- Long Survival and Prolonged Remission after Surgery and Chemotherapy in a Metastatic Mismatch Repair Deficient Pancreatic Neuroendocrine Carcinoma with MLH1/PMS2 Immunodeficiency and Minimal Microsatellite Shift.Endocrine pathology · 2020Article
- Efficacy of neoadjuvant endocrine therapy in patients with poorly differentiated neuroendocrine carcinoma of the breast: A case report.Medicine · 2020Article
- Homogeneous MMR Deficiency Throughout the Entire Tumor Mass Occurs in a Subset of Colorectal Neuroendocrine Carcinomas.Endocrine pathology · 2020Article
- Neuroendocrine Neoplasms Associated with Germline Pathogenic Variants in the Homologous Recombination Pathway.Endocrine pathology · 2019Article
- Genetics and Epigenetics of Gastroenteropancreatic Neuroendocrine Neoplasms.Endocrine reviews · 2019Review
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Some pancreatic neuroendocrine tumors (P-NETs) are associated with hereditary syndromes. An association between Lynch syndrome (LS) and P-NETs has been suggested, however it has not been confirmed to date. We describe the first case associating LS and P-NETs. Here we report a 65-year-old woman who in the past 20 years presented two colorectal carcinomas (CRC) endometrial carcinoma (EC), infiltrating ductal breast carcinoma, small intestine adenocarcinoma, two non-functioning P-NETs and sebomatricoma. With the exception of one P-NET, all these conditions were associated with LS, as confirmed by immunohistochemistry (IHC) and polymerase chain reaction (PCR). LS is caused by a mutation of a mismatch repair (MMR) gene which leads to a loss of expression of its protein. CRC is the most common tumor, followed by EC. Pancreatic tumors have also been associated with LS. Diagnosis of LS is based on clinical criteria (Amsterdam II and Bethesda) and genetic study (MMR gene mutation). The association between LS and our patient's tumors was confirmed by IHC (loss of expression of proteins MLH1 and its dimer PMS2) and the detection of microsatellite instability (MSI) using PCR.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.