ArticleThe American journal of pathology2001
PLAG1 alterations in lipoblastoma: involvement in varied mesenchymal cell types and evidence for alternative oncogenic mechanisms.
Article in The American journal of pathology, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 2 of them syntheses that pooled it.
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Who cites it
37 citing papers in PubMed, 2 syntheses or guidelines pooled it, 136 citations in OpenAlex.
- Follow-up outcomes of pediatric patients who underwent surgical resection for lipoblastomas or lipoblastomatosis: a single-institution experience with a systematic review and meta-analysis.Pediatric surgery international · 2020Pooled it
- Gene translocations in musculoskeletal neoplasms.Clinical orthopaedics and related research · 2008Pooled it
- Adolescent Paraspinal Lipoblastoma: A Case Report.Journal of orthopaedic case reports · 2026Article
- Myxoid Lipoblastoma with New Fusion TranscriptInternational journal of molecular sciences · 2026Article
- Lipoblastoma of the chest wall: a case report of successful management of a rare cause of benign chest wall swelling.Annals of medicine and surgery (2012) · 2026Article
- The role of molecular pathology in soft tissue tumor diagnosis: what the radiologist needs to know.Skeletal radiology · 2025Review
- A case of lipoblastoma in a pediatric patient.Radiology case reports · 2024Article
- Article
- Chromothripsis in lipoblastoma: second reported case with complex PLAG1 rearrangement.Molecular cytogenetics · 2023Article
- Lipoblastoma Arising in the Head and Neck: A Clinicopathologic Analysis of 20 Cases.Head and neck pathology · 2023Review
- A molecular reappraisal of matrix-producing breast metaplastic carcinoma highlighted by PLAG1 and MYC rearrangements.Tumori · 2023Article
- Lipoblastoma in one adult and 35 pediatric patients: Retrospective analysis of 36 cases.Experimental and therapeutic medicine · 2023Article
- Update of Pediatric Lipomatous Lesions: A Clinicopathological, Immunohistochemical and Molecular Overview.Journal of clinical medicine · 2022Review
- NewInternational journal of clinical and experimental pathology · 2022Article
- Lipoblastoma: Diagnosis and surgical considerations.Experimental and therapeutic medicine · 2021Article
- Lipoblastoma: computed tomographic and magnetic resonance imaging features correlate with tumor behavior and pathology.Pediatric radiology · 2021Article
- Pediatric fibromyxoid soft tissue tumor with PLAG1 fusion: A novel entity?Genes, chromosomes & cancer · 2021Article
- Lipoblastomas presenting in older children and adults: analysis of 22 cases with identification of novel PLAG1 fusion partners.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2021Article
- Letter to the Editor concerning: ‟Follow-up outcomes of pediatric patients who underwent surgical resection for lipoblastomas or lipoblastomatosis: a single-institution experience with a systematic review and meta-analysis".Pediatric surgery international · 2020Article
- A Giant Childhood Mesenteric Lipoblastoma With Extensive Maturation.Frontiers in pediatrics · 2020Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Lipoblastomas are rare soft tissue tumors that occur primarily in young children. They typically contain variably differentiated adipocytes, primitive mesenchymal cells, myxoid matrix, and fibrous trabeculae. Abnormalities in chromosome 8, leading to rearrangements of the PLAG1 gene, were demonstrated recently in four lipoblastomas. In the present report, we determine the frequency of PLAG1 alterations in 16 lipoblastomas from children aged 13 years or younger, and we also evaluate the stages of lipoblastoma differentiation at which PLAG1 genomic alterations are found. Eleven lipoblastomas (69%), including those with either classic or lipoma-like histology, had rearrangements of the 8q12 PLAG1 region. Another three lipoblastomas had polysomy for chromosome 8 in the absence of PLAG1 rearrangement. Only two cases (13%) lacked a chromosome 8 abnormality. Notably, the lipoblastomas with chromosome 8 polysomy had up to five copies of chromosome 8 as an isolated cytogenetic finding in an otherwise diploid cell. We also demonstrate that PLAG1 alterations are found in a spectrum of mesenchymal cell types in lipoblastomas, including lipoblasts, mature adipocytes, primitive mesenchymal cells, and fibroblast-like cells. This finding is consistent with neoplastic origin in a primitive mesenchymal precursor and with variable differentiation to a mature adipocyte end-point. Hence, our studies provide biological validation for the clinical observation that lipoblastomas can evolve into mature, lipoma-like, lesions. They also suggest that PLAG1 dosage alterations caused by polysomy 8 might represent an alternative oncogenic mechanism in lipoblastoma.
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