Non-ossifying fibroma: A RAS-MAPK driven benign bone neoplasm

The Journal of Pathology
Judith Vmg Bovée, Pancras Cw Hogendoorn

Abstract

Non-ossifying fibroma (NOF) has been an intriguing entity since its first description. It is the most common bone tumour, is usually asymptomatic affecting children and adolescents, is composed of a heterogeneous cell population, and undergoes spontaneous regression after puberty. In a recent article in The Journal of Pathology, Baumhoer and colleagues demonstrate mutations activating the RAS-MAPK pathway (KRAS, FGFR1 and NF1) in ∼80% of the tumours. Activation of the RAS-MAPK pathway by somatic mutations is found in a plethora of tumour types, both benign and malignant, while germline mutations cause a wide range of syndromes collectively termed the RASopathies. Their findings indicate that NOF, for long thought to be reactive, should be considered a true neoplasm. Moreover, their data suggest that only a subset of cells in the lesion contain the mutation. A second cell population consisting of histiocytes and osteoclast-like giant cells appears to be reactive. This intimate relation between WT and mutant cells is also frequently encountered in other benign and locally aggressive bone tumours and seems essential for tumourigenesis. The spontaneous regression remains enigmatic and it is tempting to speculate that pubertal hormo...Continue Reading

References

Jan 1, 1974·Clinical Orthopaedics and Related Research·D B DrennanJ J Fahey
Jan 13, 2006·Proceedings of the National Academy of Sciences of the United States of America·Robert B WestMatt van de Rijn
Sep 5, 2008·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Ramses G ForsythPancras C W Hogendoorn
Jan 19, 2010·Proceedings of the National Academy of Sciences of the United States of America·Kevin B JonesVal C Sheffield
Apr 10, 2016·Journal of the National Cancer Institute·Shumei KatoRazelle Kurzrock
Dec 13, 2016·Current Genetic Medicine Reports·William E Tidyman, Katherine A Rauen
May 16, 2017·The American Journal of Surgical Pathology·Fernanda AmaryAdrienne M Flanagan
Nov 6, 2018·Nature Communications·Carolina Cavalieri GomesNada Jabado
Dec 15, 2018·The Journal of Pathology·Daniel BaumhoerWolfgang Hartmann

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Citations

Nov 11, 2019·The Journal of Pathology·Carolina C GomesRicardo S Gomez
Dec 16, 2019·Virchows Archiv : an International Journal of Pathology·Arjen H G ClevenDaniel Baumhoer
Nov 26, 2020·Orthopaedic Nursing·Patrick Graham
Nov 26, 2019·European Journal of Medicinal Chemistry·Feng-Tao LiuZhi-Hao Shi
Jan 23, 2021·Advances in Anatomic Pathology·Joon Hyuk Choi, Jae Y Ro

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Methods Mentioned

BETA
exome sequencing

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