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The impact of changes in gadolinium-enhancement on disease progression in children with neurofibromatosis type 1-associated optic pathway glioma: a retrospective analysis

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Abstract

Purpose

There has been limited investigation of imaging features associated with visual acuity (VA) decline and initiation of treatment for patients with neurofibromatosis type 1 (NF1) and optic pathway glioma (OPG).

Methods

To evaluate the association of increased gadolinium enhancement with decline in VA, initiation of chemotherapy, and tumor growth, we performed a retrospective cohort study of children diagnosed with NF1-OPG between January 2006 to June 2016. Two cohorts were defined: a new diagnosis and a longitudinal cohort. Outcomes were examined at 1 and 2 years from initial diagnosis, and 1 and 2 years from initial increase in enhancement in the longitudinal cohort.

Results

Eighty patients were eligible; all 80 contributed to the new diagnosis cohort and 73 to the longitudinal cohort. Fifty-six patients (70%) demonstrated enhancing NF1-OPG at diagnosis. 39% of patients in the new diagnosis cohort and 45% of those in the longitudinal cohort developed increased enhancement during the study period. There was no significant association between increases in enhancement and VA decline in the newly diagnosed or longitudinal cohorts, as well as with initiation of treatment in the longitudinal cohort. Although there was an association of enhancement increase with treatment in the new diagnosis cohort, this association was not maintained when stratified by concurrent change in tumor size.

Conclusion

Increased gadolinium-enhancement independent of a concurrent increase in tumor size on MRI should not be used as a marker of NF1-OPG progression and does not appear to be associated with visual decline or initiation of chemotherapy.

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Data availability

The datasets generated during and/or analyzed during the current study may be available from the corresponding author on reasonable request.

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Acknowledgements

The authors thank Charnice Hale, Franziska Mbonglou, and Omololu Omoteso for their contributions to data acquisition.

Funding

The authors declare that no funds, grants, or other support were received for the preparation of this manuscript.

Author information

Author notes

  1. Stephanie J. Si

    Present address: University of Hawai’i Cancer Center, University of Hawai’i at Manoa, Honolulu, HI, USA

  2. Philippe Caminada

    Present address: Department of Neonatology, Children’s Hospital of Aarau, Aarau, Switzerland

  3. Francesco Maria Lo Russo

    Present address: Department of Neuroradiology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

  4. Chelsea Kotch and Stephanie J. Si co-first authors.

Authors and Affiliations

  1. Division of Oncology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Chelsea Kotch, Stephanie J. Si, Kavita Desai, Philippe Caminada, Yimei Li & Michael J. Fisher

  2. Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Chelsea Kotch, Kavita Desai & Michael J. Fisher

  3. Division of Radiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Francesco Maria Lo Russo

  4. Department of Biostatistics, Epidemiology and Informatics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Yimei Li

  5. Division of Ophthalmology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Grant T. Liu & Robert A. Avery

  6. Departments of Ophthalmology and Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Grant T. Liu & Robert A. Avery

  7. Hub for Clinical Collaboration, 3500 Civic Center Blvd, Philadelphia, PA, 19104, USA

    Chelsea Kotch

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Contributions

All authors contributed to the study. MJF and SJS. conceptualized and designed the study with input from YL, GTL, and RAA. Material preparation and data collection were performed by CK, SJS, KD, PC, and FMLR. Analyses were performed by CK, SJS, PC, FMLR, YL, and MJF. The first draft of the manuscript was written by CK. All authors critically reviewed the manuscript and have approved the final version.

Corresponding author

Correspondence to Chelsea Kotch.

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The authors declare no competing interests.

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Institutional review board approval was granted by the Children’s Hospital of Philadelphia and waiver of consent was obtained.

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Kotch, C., Si, S.J., Desai, K. et al. The impact of changes in gadolinium-enhancement on disease progression in children with neurofibromatosis type 1-associated optic pathway glioma: a retrospective analysis. J Neurooncol 164, 741–747 (2023). https://doi.org/10.1007/s11060-023-04468-3

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  • DOI: https://doi.org/10.1007/s11060-023-04468-3

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