ArticleThe Journal of pathology2019
Molecular alterations associated with metastases of solid pseudopapillary neoplasms of the pancreas.
Article in The Journal of pathology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 50 citations in OpenAlex.
- Article
- Solid pseudopapillary neoplasm of the pancreas with postoperative multiple liver metastases in an older woman: a case report.Journal of gastrointestinal oncology · 2026Article
- Development and validation of a nomogram for overall survival in pancreatic solid pseudopapillary neoplasm: a population-based study.Scientific reports · 2026Article
- Clinicopathological and molecular features of solid pseudopapillary neoplasms: a retrospective series including a small subset of aggressive cases.Pathology oncology research : POR · 2026Article
- Sex hormone receptor signaling networks drive pancreatic solid-pseudopapillary neoplasm pathogenesis and therapy.Discover oncology · 2025Review
- Proteomics in pancreatic cancer.Biomarker research · 2025Review
- Emerging pathological diagnostic strategies for solid pseudopapillary neoplasm of the pancreas: insights from omics and innovative techniques.The journal of pathology. Clinical research · 2025Review
- Circulating tumor DNA in cholangiocarcinoma: current clinical applications and future perspectives.Frontiers in cell and developmental biology · 2025Review
- Primary Solid Pseudopapillary Tumor of the Ovary: A Case Report and Review of the Literature.Journal of clinical medicine · 2024Article
- Solid pseudopapillary neoplasm (SPN) of the pancreas: current understanding on its malignant potential and management.Discover oncology · 2024Review
- A narrative review on rare types of pancreatic cancer: should they be treated as pancreatic ductal adenocarcinomas?Therapeutic advances in medical oncology · 2024Review
- Long-term organoid culture of a small intestinal neuroendocrine tumor.Frontiers in endocrinology · 2023Article
- "Pure" hepatoid tumors of the pancreas harboring CTNNB1 somatic mutations: a new entity among solid pseudopapillary neoplasms.Virchows Archiv : an international journal of pathology · 2022Article
- The role of ubiquitination and deubiquitination in tumor invasion and metastasis.International journal of biological sciences · 2022Review
- [Rare pancreatic tumors].Der Pathologe · 2021Review
- Article
- Case Report: Targeted Therapy for Metastatic Solid Pseudopapillary Neoplasm of the Pancreas WithFrontiers in oncology · 2021Article
- Rare aggressive solid pseudopapillary neoplasm of the ovary with metastatic disease following surgical resection.BMJ case reports · 2020Article
- Article
- Malignant epithelial/exocrine tumors of the pancreas.Pathologica · 2020Review
Corrections and comments
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Authors and funding
25 authors at 6 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Solid pseudopapillary neoplasms (SPN) of the pancreas are rare, low-grade malignant neoplasms that metastasise to the liver or peritoneum in 10-15% of cases. They almost invariably present somatic activating mutations of CTNNB1. No comprehensive molecular characterisation of metastatic disease has been conducted to date. We performed whole-exome sequencing and copy-number variation (CNV) analysis of 10 primary SPN and comparative sequencing of five matched primary/metastatic tumour specimens by high-coverage targeted sequencing of 409 genes. In addition to CTNNB1-activating mutations, we found inactivating mutations of epigenetic regulators (KDM6A, TET1, BAP1) associated with metastatic disease. Most of these alterations were shared between primary and metastatic lesions, suggesting that they occurred before dissemination. Differently from mutations, the majority of CNVs were not shared among lesions from the same patients and affected genes involved in metabolic and pro-proliferative pathways. Immunostaining of 27 SPNs showed that loss or reduction of KDM6A and BAP1 expression was significantly enriched in metastatic SPNs. Consistent with an increased transcriptional response to hypoxia in pancreatic adenocarcinomas bearing KDM6A inactivation, we showed that mutation or reduced KDM6A expression in SPNs is associated with increased expression of the HIF1α-regulated protein GLUT1 at both primary and metastatic sites. Our results suggest that BAP1 and KDM6A function is a barrier to the development of metastasis in a subset of SPNs, which might open novel avenues for the treatment of this disease. © 2018 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.
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