Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Mar 29;43(1):32.
doi: 10.1186/s13052-017-0353-0.

Denosumab treatment for progressive skull base giant cell tumor of bone in a 14 year old female - a case report and literature review

Samvel Bardakhchyan  1   2   3 , Leo Kager  4   5 , Samvel Danielyan  1   2   3 , Armen Avagyan  2   3 , Nerses Karamyan  1   6 , Hovhannes Vardevanyan  3   7 , Sergey Mkhitaryan  1   2   3 , Ruzanna Papyan  1   2   3 , Davit Zohrabyan  1   2   3 , Liana Safaryan  1   2   3 , Lilit Sargsyan  1   2   3 , Lilit Harutyunyan  2   3 , Lusine Hakobyan  1   2   3 , Samvel Iskanyan  2   3 , Gevorg Tamamyan  8   9   10
Affiliations
Review

Denosumab treatment for progressive skull base giant cell tumor of bone in a 14 year old female - a case report and literature review

Samvel Bardakhchyan et al. Ital J Pediatr. .

Abstract

Background: Giant cell tumor of bone (GCT) is a rare primary bone tumor, which can metastasize and undergo malignant transformation. The standard treatment of GCT is surgery. In patients with unresectable or metastatic disease, additional therapeutic options are available. These include blocking of the receptor activator of NF-kappa B ligand (RANKL) signaling pathway, which plays a role in the pathogenesis of GCT of bone, via the anti-RANKL monoclonal antibody denosumab.

Case presentation: Herein we report on a female teenager who presented in a very poor clinical condition (cachexia, diplopia, strabismus, dysphonia with palsy of cranial nerves V, VI, VIII, IX, X, XI and XII) due to progressive disease, after incomplete resection and adjuvant radiotherapy, of a GCT which affected the cervical spine (C1 and C2) as well as the skull base; and who had an impressive clinical response to denosumab therapy. To the best of our knowledge, this is the youngest patient ever reported with a skull base tumor treated with denosumab.

Conclusion: In situations when surgery can be postponed and local aggressiveness of the tumor does not urge for acute surgical intervention, upfront use of denosumab in order to reduce the tumor size might be considered. Principally, the goal of denosumab therapy is to reduce tumor size as much as possible, with the ultimate goal to make local surgery (or as in our case re-surgery) amenable. However, improvement in quality of life, as demonstrated in our patient, is also an important aspect of such targeted therapies.

Keywords: Denosumab; Giant cell tumor of bone; Skull base.

PubMed Disclaimer

References

    1. Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone. 2002
    1. Werner M. Giant cell tumour of bone: morphological, biological and histogenetical aspects. Int Orthop. 2006;30(6):484–489. doi: 10.1007/s00264-006-0215-7. - DOI - PMC - PubMed
    1. Karras NA, Polgreen LE, Ogilvie C, Manivel JC, Skubitz KM, Lipsitz E. Denosumab treatment of metastatic giant-cell tumor of bone in a 10-year-Old girl. J Clin Oncol. 2013;31(12):e200–e202. doi: 10.1200/JCO.2012.46.4255. - DOI - PMC - PubMed
    1. Thomas D, Skubitz K. Giant cell tumour of bone. Curr Opin Oncol. 2009;21(4):338–44. doi: 10.1097/CCO.0b013e32832c951d. - DOI - PubMed
    1. Miller IJ, Blank A, Yin SM, et al. A case of recurrent giant cell tumor of bone with malignant transformation and benign pulmonary metastases. Diagn Pathol. 2010;5(1):62. doi: 10.1186/1746-1596-5-62. - DOI - PMC - PubMed