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Survival and prognosis in malignant giant cell tumor of bone: A population-based analysis from 1984 to 2013.

ABSTRACT: Background:Malignant giant cell tumor of bone (MGCTB) is extremely rare. Currently, population-based prognosis studies are lacking. This study aimed to determine the impact of demographics, tumor characteristics, and treatment on prognosis among patients with MGCTB. Methods:The Surveillance, Epidemiology, and End Results database was used to identify patients with MGCTB from 1984 to 2013. Kaplan-Meier analyses were performed to determine the overall survival (OS). Univariable and multivariable Cox analyses were conducted to identify prognostic factors. Results:There were 250 patients with MGCTB included in our study. The multivariate Cox analysis revealed that age at diagnosis (hazard ratio [HR]: 1.09; 95% confidence interval [CI]: 1.07-1.11; P < 0.001), tumor size (HR: 7.04; 95% CI: 2.38-20.77; P < 0.001), tumor extension (regional vs. localized, HR: 2.64; 95% CI: 1.10-6.34; P?=?0.030; distant vs. localized, HR: 6.12; 95% CI: 2.27-16.49; P < 0.001), and radiotherapy (HR: 0.41; 95% CI: 0.18-0.89; P?=?0.025) were independent risk factors of OS in patients with MGCTB. Notably, tumor site (HR: 1.98; 95% CI: 0.99-4.00; P?=?0.055) exhibited borderline significance. Additionally, we found that patients with tumors measuring >70?mm (P?=?0.015), located in the axial skeleton (P < 0.001) and presented with distant metastasis (P < 0.001) tended to receive radiotherapy. Moreover, a nomogram model integrating independent predictors was established to estimate the OS of patients with MGCTB. Conclusion:This study provides a population-based assessment of the largest number of patients with MGCTB. We found that older age, larger tumor size, regional or distant metastasis, and lack of radiotherapy was associated with poor OS. Surgical methods were not significantly associated with OS. Furthermore, we built a high-quality nomogram to predict 1-, 3-, and 5-year OS for patients with MGCTB. These findings may assist in the clinical diagnosis and treatment of MGCTB.

SUBMITTER: Lin JL

PROVIDER: S-EPMC6812025 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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Survival and prognosis in malignant giant cell tumor of bone: A population-based analysis from 1984 to 2013.

Lin Jia-Liang JL Wu Yu-Hao YH Shi Yi-Feng YF Lin Hao H Nisar Majid M Meftah Zaher Z Xu Cong C Chen Jiao-Xiang JX Wang Xiang-Yang XY

Journal of bone oncology 20190911

<h4>Background</h4>Malignant giant cell tumor of bone (MGCTB) is extremely rare. Currently, population-based prognosis studies are lacking. This study aimed to determine the impact of demographics, tumor characteristics, and treatment on prognosis among patients with MGCTB.<h4>Methods</h4>The Surveillance, Epidemiology, and End Results database was used to identify patients with MGCTB from 1984 to 2013. Kaplan-Meier analyses were performed to determine the overall survival (OS). Univariable and ...[more]

PMID: 31667061

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Project description:The rare benign giant cell tumour of bone (GCTB) is defined by an almost unique mutation in the H3.3 family of histone genes H3-3A or H3-3B; however, the same mutation is occasionally found in primary malignant bone tumours which share many features with the benign variant. Moreover, lung metastases can occur despite the absence of malignant histological features in either the primary or metastatic lesions. Herein we investigated the genetic events of 17 GCTBs including benign and malignant variants and the methylation profiles of 122 bone tumour samples including GCTBs. Benign GCTBs possessed few somatic alterations and no other known drivers besides the H3.3 mutation, whereas all malignant tumours harboured at least one additional driver mutation and exhibited genomic features resembling osteosarcomas, including high mutational burden, additional driver event(s), and a high degree of aneuploidy. The H3.3 mutation was found to predate the development of aneuploidy. In contrast to osteosarcomas, malignant H3.3-mutated tumours were enriched for a variety of alterations involving TERT, other than amplification, suggesting telomere dysfunction in the transformation of benign to malignant GCTB. DNA sequencing of the benign metastasising GCTB revealed no additional driver alterations; polyclonal seeding in the lung was identified, implying that the metastatic lesions represent an embolic event. Unsupervised clustering of DNA methylation profiles revealed that malignant H3.3-mutated tumours are distinct from their benign counterpart, and other bone tumours. Differential methylation analysis identified CCND1, encoding cyclin D1, as a plausible cancer driver gene in these tumours because hypermethylation of the CCND1 promoter was specific for GCTBs. We report here the genomic and methylation patterns underlying the rare clinical phenomena of benign metastasising and malignant transformation of GCTB and show how the combination of genomic and epigenomic findings could potentially distinguish benign from malignant GCTBs, thereby predicting aggressive behaviour in challenging diagnostic cases. © 2020 The Authors. The Journal of Pathology published by John Wiley & Sons, Ltd. on behalf of The Pathological Society of Great Britain and Ireland.

Dataset Information

Survival and prognosis in malignant giant cell tumor of bone: A population-based analysis from 1984 to 2013.

Publications

Survival and prognosis in malignant giant cell tumor of bone: A population-based analysis from 1984 to 2013.

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