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Surgical Management and Denosumab for Aneurysmal Bone Cysts of the Spine in an Australian Tertiary Paediatric Centre

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Abstract

Aneurysmal bone cysts (ABC) are rare osteolytic, benign but often locally aggressive tumours of the long bones or vertebrae. For spinal ABC, surgical management, embolisation or sclerotherapy alone often carry high morbidity and/or high recurrence rates. Interruption of receptor activator of nuclear factor-kappa B ligand (RANKL) signalling holds promise as an effective therapeutic strategy for these tumours. We aimed to review the approach to surgical management and evaluate the efficacy and safety of denosumab for ABC of the spine in children. Retrospective review of 7 patients treated with denosumab using a standardised protocol for ABC of the spine in a tertiary paediatric centre. Surgical intervention was only conducted if there was spinal instability or significant neurological impairment. Denosumab 70 mg/m2 was given 4-weekly for at least 6 months, followed by 2 doses of zoledronate 0.025 mg/kg, aiming to prevent rebound hypercalcaemia. All patients achieved stability of the spine and resolution of neurological impairment, if present. Six patients achieved metabolic remission and have ceased denosumab without recurrence to date; the other showed clinical and radiological improvement without complete metabolic remission. Three patients developed symptomatic hypercalcaemia 5–7 months after cessation of denosumab, requiring additional bisphosphonate treatment. We present our algorithm for the surgical and medical management of paediatric spinal ABC. Denosumab produced a radiological and metabolic response in all patients, with complete remission in most. Follow-up time was not long enough to evaluate the endurance of response after cessation in some patients. Incidence of rebound hypercalcaemia in this paediatric cohort was high, prompting a change to our protocol.

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Acknowledgements

Other members of Denosumab Treatment Group: Brian Hsu, Nathan Hartin, Vinay Kulkarni, David Lord, Richard Widmer.

Funding

No external funding was received for this project.

Author information

Author notes

  1. Joel A. Vanderniet and Dionysios Tsinas have contributed equally to this work.

Authors and Affiliations

  1. Institute of Endocrinology and Diabetes, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, Sydney, NSW, 2145, Australia

    Joel A. Vanderniet, Christie-Lee Wall, Myra Poon & Andrew Biggin

  2. Department of Orthopaedics, The Children’s Hospital at Westmead, Sydney, NSW, Australia

    Dionysios Tsinas, Corinne Keane & Randolph J. Gray

  3. Faculty of Medicine and Health, University of Sydney, Sydney, NSW, Australia

    Christian M. Girgis, Bhavna Padhye, Andrew Biggin & Luciano Dalla-Pozza

  4. Department of Endocrinology, Royal North Shore Hospital, Sydney, NSW, Australia

    Christian M. Girgis

  5. Department of Diabetes and Endocrinology, Westmead Hospital, Sydney, NSW, Australia

    Christian M. Girgis

  6. Department of Nuclear Medicine, The Children’s Hospital at Westmead, Sydney, NSW, Australia

    Kevin London & Julie Briody

  7. Department of Dentistry, The Children’s Hospital at Westmead, Sydney, NSW, Australia

    Sally Hibbert

  8. Department of Paediatric Dentistry, Westmead Centre for Oral Health, Sydney, NSW, Australia

    Sally Hibbert

  9. Cancer Centre for Children, The Children’s Hospital at Westmead, Sydney, NSW, Australia

    Bhavna Padhye & Luciano Dalla-Pozza

  10. Child Health Research Centre, The University of Queensland, Brisbane, QLD, Australia

    Craig F. Munns

  11. Mayne Academy of Paediatrics, The University of Queensland, Brisbane, QLD, Australia

    Craig F. Munns

Authors
  1. Joel A. Vanderniet
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  2. Dionysios Tsinas
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  3. Christie-Lee Wall
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  8. Sally Hibbert
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  12. Luciano Dalla-Pozza
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  14. Craig F. Munns
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Contributions

JAV: conceptualisation, methodology, data collection, analysis, writing—original draft; DT: conceptualisation, data collection, analysis, writing—original draft; CLW: conceptualisation, methodology, data collection, writing—review & editing; CMG: data collection, writing—review & editing; KL: writing—review & editing; CK—writing—review & editing; SH: writing—review & editing; JB: data curation, writing—review & editing; MP: writing—review & editing; BP: writing—review & editing; AB: visualisation, writing—review & editing; LDP: writing—review & editing; RJG: conceptualisation, writing—review & editing, supervision; CFM: conceptualisation, methodology, writing—review & editing, supervision.

Corresponding author

Correspondence to Joel A. Vanderniet.

Ethics declarations

Denosumab was administered following approval from the Sydney Children's Hospitals Network Drug Committee.

Conflict of interest

Joel A. Vanderniet, Dionysios Tsinas, Christie-Lee Wall, Christian M. Girgis, Kevin London, Corinne Keane, Julie Briody, Sally Hibbert, Myra Poon, Bhavna Padhye, Andrew Biggin, Luciano Dalla-Pozza, Randolph J. Gray and Craig F. Munns have no conflicts of interest to declare.

Ethical approval

This study was approved by the Human Research Ethics Committee at our institution (2019/ETH13024) and conducted in accordance with the ethical standards of the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent was not required for this retrospective review of clinical data, in which no identifying details were published.

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Vanderniet, J.A., Tsinas, D., Wall, CL. et al. Surgical Management and Denosumab for Aneurysmal Bone Cysts of the Spine in an Australian Tertiary Paediatric Centre. Calcif Tissue Int 112, 592–602 (2023). https://doi.org/10.1007/s00223-023-01068-1

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