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

  • Mahendranath Moharir
  • Kevin London
  • Robert Howman-Giles
  • Kathryn North
Original Article

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.

Keywords

Neurofibromatosis type 1 PET/CT Plexiform neuroma Optic glioma Malignant peripheral nerve sheath tumour 

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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Mahendranath Moharir
    • 1
  • Kevin London
    • 2
  • Robert Howman-Giles
    • 3
  • Kathryn North
    • 4
  1. 1.Division of NeurologyThe Hospital for Sick ChildrenOntarioCanada
  2. 2.Department of Nuclear MedicineChildren’s Hospital at WestmeadSydneyAustralia
  3. 3.Discipline of Imaging, Faculty of MedicineUniversity of SydneySydneyAustralia
  4. 4.Institute for Neuroscience and Muscle ResearchChildren’s Hospital at WestmeadSydneyAustralia

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