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Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1

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Abstract

Purpose

There is little consensus regarding optimal surveillance of optic pathway glioma (OPG) and plexiform neurofibroma (PNF) in childhood neurofibromatosis type 1 (NF1). 18F-2-Fluoro-2-deoxy-D-glucose (FDG) positron emission tomography and computed tomography (PET/CT) is employed in the surveillance of adult PNFs; but its utility has neither been specifically studied in children with PNFs nor in children with OPG.

Methods

Review of PET/CT studies was performed in NF1 children with OPG or PNF. FDG-avidity of tumours was semi-quantitatively analysed and graded by calculating the maximum standardised uptake value (SUVmax) [grade 1: <3 (low), grade 2: >3–<4 (intermediate), grade 3: >4 (intense)].

Results

Eighteen children (ten girls; median age: 8.5–years) had PET/CT. Nineteen OPGs were imaged. The SUVmax could be measured in 16. Ten were grade 1 and three each were grade 2 and grade 3. FDG-avidity reduced from grade 3 to grade 1 in two symptomatic OPGs following chemotherapy and this was associated with clinical improvement. PET/CT diagnosed symptomatic OPGs with a sensitivity of 0.625 [95% confidence interval (CI): 0.259–0.897] and specificity of 0.875 (95% CI: 0.466–0.993). Sixteen PNFs were imaged. Twelve were grade 1 and two each were grade 2 and grade 3. The two grade 3 PNFs were confirmed malignant peripheral nerve sheath tumours. PET/CT diagnosed malignant transformation with a sensitivity of 1.0 (95% CI: 0.197–1.0) and specificity of 0.857 (95% CI: 0.561–0.974).

Conclusion

PET/CT may contribute useful information to the surveillance of OPG in childhood NF1—particularly to identify progressive, symptomatic tumours. As in adults, PET/CT is useful for the detection of malignant transformation in PNFs in children with NF1.

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Authors and Affiliations

  1. Division of Neurology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada

    Mahendranath Moharir

  2. Department of Nuclear Medicine, Children’s Hospital at Westmead, Sydney, NSW 2145, Australia

    Kevin London

  3. Discipline of Imaging, Faculty of Medicine, University of Sydney, Sydney, Australia

    Robert Howman-Giles

  4. Institute for Neuroscience and Muscle Research, Children’s Hospital at Westmead, Sydney, NSW, 2145, Australia

    Kathryn North

Authors
  1. Mahendranath Moharir
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  2. Kevin London
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  3. Robert Howman-Giles
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  4. Kathryn North
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Correspondence to Kathryn North.

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Moharir, M., London, K., Howman-Giles, R. et al. Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging 37, 1309–1317 (2010). https://doi.org/10.1007/s00259-010-1386-4

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  • DOI: https://doi.org/10.1007/s00259-010-1386-4

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