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01.12.2017 | Case report | Ausgabe 1/2017 Open Access

Italian Journal of Pediatrics 1/2017

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

Zeitschrift:
Italian Journal of Pediatrics > Ausgabe 1/2017
Autoren:
Samvel Bardakhchyan, Leo Kager, Samvel Danielyan, Armen Avagyan, Nerses Karamyan, Hovhannes Vardevanyan, Sergey Mkhitaryan, Ruzanna Papyan, Davit Zohrabyan, Liana Safaryan, Lilit Sargsyan, Lilit Harutyunyan, Lusine Hakobyan, Samvel Iskanyan, Gevorg Tamamyan
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​s13052-017-0353-0) contains supplementary material, which is available to authorized users.

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.
Zusatzmaterial
Additional file 1: Figure S1. MRI at diagnosis. (A) axial, (B) coronal and (C) sagittal section of T1 weighted MRI after contrast administration revealing an intra-extracranial pathologic formation of craniovertebral region 4,4×5,5×5,2 cm in size, which was rapidly enhancing after the gadolinium injection, with decay of bones of craniovertebral region, upper third of processus odontoideus of C2 vertebrae, luxation of cervical vertebrae at the level of atlantoaxial articulation, more profoundly on the right side. Formation grows into the sphenoid bone reaching the brainstem, largely the pons, constrictes foramen of Magendie, compresses the left cerebellar hemisphere and reaches the nasopharyngeal region without entering nasopharyngeal space. Figure S2. MRI after radiotherapy. (A) axial, (B) coronal and (C) sagittal images of T1 weighted MRI after contrast admision performed after surgery and radiation therapy revealed recurrence of the pathologic mass of the craniovertebral junction, up to 5,7×7,1×6,0 cm in size, with central necrosiss. Signs of decay, which replaces clivus, ventral parts of C1-C2, and condyles of temporal bone. Ventrally it infiltrates retropharyngeal space, dorsally deformates and obliterates lumen of pontine cistern and cisterna magna, compressing ventral parts of brainstem. Figure S3. MRI after 4 cycles of denosumab. (A) axial, (B) coronal and (C) sagittal images of T1 weighted MRI after contrast administration with image subtraction (C) after 4 cycles of denosumab treatment revealing an intra-extracranial pathologic formation of craniovertebral region 3,2×6,8×5,2 cm in size, with decay of bones of atlantooccipital and atlantoaxial articulation and upper third of processus odontoideus of C2 vertebrae, with spine offset to the right. The pathological mass enhances after the gadolinium injection, with small hypointense areas, with indicate focal necrosis. The mass grows into the sphenoid bone reaching the brainstem, largely the pons, ventrally reaches nasopharyngeal region without entering nasopharyngeal space. Dorsolateral parts of formation reach condyles of temporal bone, constricts craniovertebral transition and deformates front loop of medulla. The formation is inhomogen due to small parts of cystic transformation. (DOCX 688 kb)
13052_2017_353_MOESM1_ESM.docx
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