ArticleClinical cancer research : an official journal of the American Association for Cancer Research2024
DICER1-Related Tumor Predisposition: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Article in Clinical cancer research : an official journal of the American Association for Cancer Research, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed.
- Surgical management and molecular diagnosis of pediatric cystic nephroma: a single-center case series with implications forTranslational andrology and urology · 2026Article
- Childhood cancer predisposition study: a prospective registry and biorepository protocol.BMJ open · 2026Article
- Clinical Impact of Germline Multigene Sequencing in Pediatric Cohorts with a Wide Spectrum of Neoplasms.International journal of molecular sciences · 2026Article
- The New Tumor Predisposition Syndromes with Neuro-Oncological Relevance-A Comprehensive Review for Neuroradiologists.Clinical neuroradiology · 2026Review
- Pleuropulmonary Blastoma and Diagnostic Pitfalls: A Report from the International Pleuropulmonary Blastoma/DICER1 Registry.Archives of pathology & laboratory medicine · 2026Article
- Inflammatory and Nested Testicular Sex Cord Tumors: Clinical and Molecular Characterization.Genes · 2026Review
- Developing a multidisciplinary pediatric cancer predisposition service at a mid-sized children's hospital.Frontiers in oncology · 2026Article
- Double jeopardy: howFrontiers in cell and developmental biology · 2026Review
- Case Report: Congenital pulmonary airway malformation associated with a germline DICER1 splicing variant.Frontiers in pediatrics · 2026Article
- Large pleuropulmonary blastoma mimicking congenital pulmonary airway malformation in an infant: A case report.Respiratory medicine case reports · 2026Article
- Expanding the Molecular Data ofHuman mutation · 2026Article
- Unmasking familial follicular cell-derived thyroid neoplasms associated with syndromes: DICER1 and PTEN-hamartoma tumor syndromes.Virchows Archiv : an international journal of pathology · 2026Review
- Mapping the Natural History of Benign DICER1-Related Lesions and Identifying Predictors of Malignancy.Fortune journal of health sciences · 2026Article
- DICER1 and DGCR8 in thyroid tumorigenesis: miRNA biogenesis and histopathologic diversity.European thyroid journal · 2025Review
- Review
- Rapid recurrence of ovarian mixed sex-cord-stromal tumor associated with DICER1 gene mutation: a case analysis and literature review.Annals of medicine and surgery (2012) · 2025Article
- Prognostic Significance of GermlineJCO precision oncology · 2025Article
- Pleuropulmonary blastoma and DICER1-related tumor predisposition: from clinicopathologic observations to clinical trial.Current opinion in pediatrics · 2025Review
- DICER1: The Argonaute Endonuclease Family Member and Its Role in Pediatric and Youth Pathology.Biology · 2025Review
- Update on Pediatric Surveillance Recommendations for PTEN Hamartoma Tumor Syndrome, DICER1-Related Tumor Predisposition, and Tuberous Sclerosis Complex.Clinical cancer research : an official journal of the American Association for Cancer Research · 2025Review
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Authors and funding
31 authors.
Funding
Abstract
purposeDICER1-related tumor predisposition increases risk for a spectrum of benign and malignant tumors. In 2018, the International Pleuropulmonary Blastoma (PPB)/DICER1 Registry published guidelines for testing- and imaging-based surveillance of individuals with a known or suspected germline DICER1 pathogenic or likely pathogenic (P/LP) variant. One of the goals of the Registry is to continue to refine these guidelines as additional data become available. EXPERIMENTAL
designIndividuals were enrolled in the International PPB/DICER1 Registry, the International Ovarian and Testicular Stromal Tumor Registry, and/or the NCI Natural History of DICER1 Syndrome study.
resultsReview of participant records identified 713 participants with a germline DICER1 P/LP variant from 38 countries. To date, 5 cases of type I and 29 cases of type Ir PPB have been diagnosed by surveillance in enrolled individuals. One hundred and three individuals with a germline P/LP variant developed a primary ovarian Sertoli-Leydig cell tumor at a median age of 14 years (range: 11 months-66 years); 13% were diagnosed before 8 years of age, the current age of onset of pelvic surveillance. Additionally, 4% of Sertoli-Leydig cell tumors were diagnosed before 4 years of age.
conclusionsOngoing data collection highlights the role of lung surveillance in the early detection of PPB and suggests that imaging-based detection and early resection may decrease the risk of advanced PPB. DICER1-related ovarian tumors were detected before 8 years of age, prompting the Registry to recommend earlier initiation of ovarian surveillance with pelvic ultrasound beginning at the time of detection of a germline DICER1 P/LP variant.
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