Next Article in Journal
Intra- and Interobserver Variability in Magnetic Resonance Imaging Measurements in Rectal Cancer Patients
Previous Article in Journal
Pirfenidone Reduces Epithelial–Mesenchymal Transition and Spheroid Formation in Breast Carcinoma through Targeting Cancer-Associated Fibroblasts (CAFs)
Previous Article in Special Issue
Current Concepts in the Treatment of Giant Cell Tumors of Bone
Review

Cell Biology of Giant Cell Tumour of Bone: Crosstalk between m/wt Nucleosome H3.3, Telomeres and Osteoclastogenesis

1
Department of Pathology, University Hospital Brussels (UZB), Laarbeeklaan 101, 1090 Brussels, Belgium
2
Labaratorium for Experimental Pathology (EXPA), Vrije Universiteit Brussel (VUB), Laarbeeklaan 103, 1090 Brussels, Belgium
3
1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Üllöi ut 26, 1085 Budapest, Hungary
4
Department of Histopathology, Nuffield Orthopaedic Centre, University of Oxford, NDORMS, Oxford OX3 7HE, UK
5
Department of Pathology, Leiden University Medical Center (LUMC), Albinusdreef 2, 2300 RC Leiden, The Netherlands
*
Author to whom correspondence should be addressed.
Academic Editors: Emanuela Palmerini and Joseph Schwab
Cancers 2021, 13(20), 5119; https://doi.org/10.3390/cancers13205119
Received: 6 September 2021 / Revised: 6 October 2021 / Accepted: 8 October 2021 / Published: 13 October 2021
(This article belongs to the Special Issue Research Advances in Giant Cell Tumor of Bone)
The overall clinical and cell biological behaviour of giant cell tumour of bone still remains very difficult to understand. This entity is a prototype neoplasm illustrating that the scientific work around nucleosome mutations in cancer is a hot topic. This review tries to summarise and integrate the wide spread insights in this research era with specific attention to the cell biological aspects of giant cell tumour of bone with the focus on genomic and epigenomic alterations that may lead to genomic instability and malignancy. Moreover, the ultimate goal is to connect all major characteristics of this tumour to a comprehensive understanding, like parts of a broad etiogenic puzzle, which may lead to new insights.
Giant cell tumour of bone (GCTB) is a rare and intriguing primary bone neoplasm. Worrisome clinical features are its local destructive behaviour, its high tendency to recur after surgical therapy and its ability to create so-called benign lung metastases (lung ‘plugs’). GCTB displays a complex and difficult-to-understand cell biological behaviour because of its heterogenous morphology. Recently, a driver mutation in histone H3.3 was found. This mutation is highly conserved in GCTB but can also be detected in glioblastoma. Denosumab was recently introduced as an extra option of medical treatment next to traditional surgical and in rare cases, radiotherapy. Despite these new insights, many ‘old’ questions about the key features of GCTB remain unanswered, such as the presence of telomeric associations (TAs), the reactivation of hTERT, and its slight genomic instability. This review summarises the recent relevant literature of histone H3.3 in relation to the GCTB-specific G34W mutation and pays specific attention to the G34W mutation in relation to the development of TAs, genomic instability, and the characteristic morphology of GCTB. As pieces of an etiogenetic puzzle, this review tries fitting all these molecular features and the unique H3.3 G34W mutation together in GCTB. View Full-Text
Keywords: giant cell tumour of bone; osteoclast; mononuclear spindle-shaped cell; telomeric association; G34W giant cell tumour of bone; osteoclast; mononuclear spindle-shaped cell; telomeric association; G34W
Show Figures

Figure 1

MDPI and ACS Style

Forsyth, R.G.; Krenács, T.; Athanasou, N.; Hogendoorn, P.C.W. Cell Biology of Giant Cell Tumour of Bone: Crosstalk between m/wt Nucleosome H3.3, Telomeres and Osteoclastogenesis. Cancers 2021, 13, 5119. https://doi.org/10.3390/cancers13205119

AMA Style

Forsyth RG, Krenács T, Athanasou N, Hogendoorn PCW. Cell Biology of Giant Cell Tumour of Bone: Crosstalk between m/wt Nucleosome H3.3, Telomeres and Osteoclastogenesis. Cancers. 2021; 13(20):5119. https://doi.org/10.3390/cancers13205119

Chicago/Turabian Style

Forsyth, Ramses G., Tibor Krenács, Nicholas Athanasou, and Pancras C.W. Hogendoorn 2021. "Cell Biology of Giant Cell Tumour of Bone: Crosstalk between m/wt Nucleosome H3.3, Telomeres and Osteoclastogenesis" Cancers 13, no. 20: 5119. https://doi.org/10.3390/cancers13205119

Find Other Styles
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Back to TopTop