ArticleProceedings of the National Academy of Sciences of the United States of America2010
Concomitant deletions of tumor suppressor genes MEN1 and AIP are essential for the pathogenesis of the brown fat tumor hibernoma.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 69 citations in OpenAlex.
- The role of molecular pathology in soft tissue tumor diagnosis: what the radiologist needs to know.Skeletal radiology · 2025Review
- Childhood Multiple Endocrine Neoplasia (MEN) Syndromes: Genetics, Clinical Heterogeneity and Modifying Genes.Journal of clinical medicine · 2024Review
- Hibernoma in the renal sinus: A case mimicking malignancy.IJU case reports · 2024Article
- Cutaneous lesions and other non-endocrine manifestations of Multiple Endocrine Neoplasia type 1 syndrome.Frontiers in endocrinology · 2023Article
- Beyond the "3 Ps": A critical appraisal of the non-endocrine manifestations of multiple endocrine neoplasia type 1.Frontiers in endocrinology · 2022Review
- Clinical aspects of multiple endocrine neoplasia type 1.Nature reviews. Endocrinology · 2021Review
- Nuclear expression of MDM2 in hibernoma: a potential diagnostic pitfall.Virchows Archiv : an international journal of pathology · 2021Article
- Article
- Different patterns of clonal evolution among different sarcoma subtypes followed for up to 25 years.Nature communications · 2018Article
- Hibernoma Mimicking Atypical Lipomatous Tumor: 64 Cases of a Morphologically Distinct Subset.The American journal of surgical pathology · 2018Article
- Extra peritoneal giant pelvic hibernoma: a case report.Journal of surgical case reports · 2017Article
- Intramuscular Hibernoma: A Rare Tumour in Buttock.Journal of clinical and diagnostic research : JCDR · 2015Article
- Intraosseous hibernoma: characterization of five cases and literature review.Skeletal radiology · 2014Review
- GRM1 is upregulated through gene fusion and promoter swapping in chondromyxoid fibroma.Nature genetics · 2014Article
- MEN1 Syndrome and Hibernoma: An Uncommonly Recognised Association?Case reports in medicine · 2014Article
- Intraosseous hibernoma: a case report and review of the literature.Skeletal radiology · 2013Review
- Familial isolated pituitary adenomas (FIPA) and the pituitary adenoma predisposition due to mutations in the aryl hydrocarbon receptor interacting protein (AIP) gene.Endocrine reviews · 2013Review
- Recurrent chromosome 22 deletions in osteoblastoma affect inhibitors of the Wnt/beta-catenin signaling pathway.PloS one · 2013Article
- Familial isolated pituitary adenomas: an emerging clinical entity.Journal of endocrinological investigation · 2012Review
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Authors and funding
9 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Hibernomas are benign tumors with morphological features resembling brown fat. They consistently display cytogenetic rearrangements, typically translocations, involving chromosome band 11q13. Here we demonstrate that these aberrations are associated with concomitant deletions of AIP and MEN1, tumor suppressor genes that are located 3 Mb apart and that underlie the hereditary syndromes pituitary adenoma predisposition and multiple endocrine neoplasia type I. MEN1 and AIP displayed a low expression in hibernomas whereas the expression of genes up-regulated in brown fat--PPARA, PPARG, PPARGC1A, and UCP1--was high. Thus, loss of MEN1 and AIP is likely to be pathogenetically essential for hibernoma development. Simultaneous loss of two tumor suppressor genes has not previously been shown to result from a neoplasia-associated translocation. Furthermore, in contrast to the prevailing assumption that benign tumors harbor relatively few genetic aberrations, the present analyses demonstrate that a considerable number of chromosome breaks are involved in the pathogenesis of hibernoma.
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