The utility of [18F]FDG PET/CT in children with neurofibromatosis type 1: A retrospective single-center study

Document Type : Original Article

Authors

1 Diagnostic Imaging Department, The Hospital for Sick Children, Toronto, Canada

2 Research Center for Nuclear Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran

3 Medical Imaging Department, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

Abstract

Introduction: Neurofibromatosis type 1 (NF1) is a prevalent inherited condition, having a risk for malignant conversion to malignant peripheral nerve sheath tumor (MPNST). Our study objective was to assess the effectiveness of [18F]FDG PET/CT and Magnetic Resonance Imaging (MR) in identifying early transformation in pediatric patients, given the limited data available in existing literature.
Methods: In this study children with suspected or confirmed NF1 who underwent [18F]FDG between January 2007 and April 2024 were included in a retrospective cross-sectional analysis. Exclusion criteria was follow-up periods shorter than one-year post [18F]FDG results.
Results: The study included 13 patients (6 females, 7 males), with a median age of 11 years, and a total of 16 lesions. Only two lesions were confirmed by biopsy to have undergone malignant transformation into a MPNST. Although the findings varied, [18F]FDG imaging was able to anticipate the malignant transformation only in one of these two cases.
Conclusions: Due to the limited sample size, definitive conclusions could not be provided. We were unable to propose a specific SUVmax cutoff to predict the malignant transformation of neurofibromas in children with NF1  disease.

Keywords

Main Subjects

References

  1. Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer. 1986 May 15;57(10):2006-21.
  2. McGaughran JM, Harris DI, Donnai D, Teare D, MacLeod R, Westerbeek R, Kingston H, Super M, Harris R, Evans DG. A clinical study of type 1 neurofibromatosis in north west England. J Med Genet. 1999 Mar;36(3):197-203.
  3. Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res. 2002 Mar 1;62(5):1573-7.
  4. Leroy K, Dumas V, Martin-Garcia N, Falzone MC, Voisin MC, Wechsler J, Revuz J, Créange A, Levy E, Lantieri L, Zeller J, Wolkenstein P. Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1: a clinicopathologic and molecular study of 17 patients. Arch Dermatol. 2001 Jul;137(7):908-13.
  5. Moharir M, London K, Howman-Giles R, North K. Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. 2010;37(7):1309-17.
  6. Warbey VS, Ferner RE, Dunn JT, Calonje E, O'Doherty MJ. [18F]FDG PET/CT in the diagnosis of malignant peripheral nerve sheath tumours in neurofibromatosis type-1. Eur J Nucl Med Mol Imaging. 2009 May;36(5):751-7. 
  7. Derlin T, Tornquist K, Münster S, Apostolova I, Hagel C, Friedrich RE, Wedegärtner U, Mautner VF. Comparative effectiveness of 18F-FDG PET/CT versus whole-body MRI for detection of malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Clin Nucl Med. 2013 Jan;38(1):e19-25.
  8. Wasa J, Nishida Y, Tsukushi S, Shido Y, Sugiura H, Nakashima H, Ishiguro N. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 2010 Jun;194(6):1568-74.
  9. Ferner RE, Lucas JD, O'Doherty MJ, Hughes RA, Smith MA, Cronin BF, Bingham J. Evaluation of (18)fluorodeoxyglucose positron emission tomography ((18)FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry. 2000 Mar;68(3):353-7.
  10. Tsai LL, Drubach L, Fahey F, Irons M, Voss S, Ullrich NJ. [18F]-Fluorodeoxyglucose positron emission tomography in children with neurofibromatosis type 1 and plexiform neurofibromas: Correlation with malignant transformation. J Neurooncol. 2012;108(3):469-75.
  11. Benz MR, Czernin J, Dry SM, Tap WD, Allen-Auerbach MS, Elashoff D, Phelps ME, Weber WA, Eilber FC. Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer. 2010 Jan 15;116(2):451-8. 
  12. Broski SM, Johnson GB, Howe BM, Nathan MA, Wenger DE, Spinner RJ, Amrami KK. Evaluation of (18)F-FDG PET and MRI in differentiating benign and malignant peripheral nerve sheath tumors. Skeletal Radiol. 2016 Aug;45(8):1097-105. 
  13. Azizi AA, Slavc I, Theisen BE, Rausch I, Weber M, Happak W, Aszmann O, Hojreh A, Peyrl A, Amann G, Benkoe TM. Monitoring of plexiform neurofibroma in children and adolescents with neurofibromatosis type 1 by [18F] FDG‐PET imaging. Is it of value in asymptomatic patients?. Pediatr Blood Cancer. 2018 Jan;65(1):e26733.
  14. Salamon J, Derlin T, Bannas P, Busch JD, Herrmann J, Bockhorn M, Hagel C, Friedrich RE, Adam G, Mautner VF. Evaluation of intratumoural heterogeneity on 18 F-FDG PET/CT for characterization of peripheral nerve sheath tumours in neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. 2013 May;40:685-92.
  15. Salamon J, Veldhoen S, Apostolova I, Bannas P, Yamamura J, Herrmann J, Friedrich RE, Adam G, Mautner VF, Derlin T. 18F-FDG PET/CT for detection of malignant peripheral nerve sheath tumours in neurofibromatosis type 1: tumour-to-liver ratio is superior to an SUVmax cut-off. Eur Radiol. 2014 Feb;24(2):405-12.
  16. Bredella MA, Torriani M, Hornicek F, Ouellette HA, Plamer WE, Williams Z, Fischman AJ, Plotkin SR. Value of PET in the assessment of patients with neurofibromatosis type 1. AJR Am J Roentgenol. 2007 Oct;189(4):928-35.
  17. Ferner RE, Golding JF, Smith M, Calonje E, Jan W, Sanjayanathan V, O’Doherty M. [ 18 F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol. 2008;19(2):390-4.
  18. Brenner W, Friedrich RE, Gawad KA, Hagel C, von Deimling A, de Wit M, Buchert R, Clausen M, Mautner VF. Prognostic relevance of FDG PET in patients with neurofibromatosis type-1 and malignant peripheral nerve sheath tumours. Eur J Nucl Med Mol Imaging. 2006 Apr;33(4):428-32.
  19. Geitenbeek RTJ, Martin E, Graven LH, Broen MPG, Anten MHME, van der Pol JAJ, Verhoef C, Taal W. Diagnostic value of 18F-FDG PET-CT in detecting malignant peripheral nerve sheath tumors among adult and pediatric neurofibromatosis type 1 patients. J Neurooncol. 2022 Feb;156(3):559-67.
  20. Yue X, Stauff E, Boyapati S, Langhans SA, Xu W, Makrogiannis S, Okorie UJ, Okorie AM, Kandula VVR, Kecskemethy HH, Nikam RM, Averill LW, Shaffer TH. PET imaging of neurofibromatosis type 1 with a Fluorine-18 labeled tryptophan radiotracer. Pharmaceuticals (Basel). 2024 May 27;17(6):685.
  21. Meany H, Dombi E, Reynolds J, Whatley M, Kurwa A, Tsokos M, Salzer W, Gillespie A, Baldwin A, Derdak J, Widemann B. 18-fluorodeoxyglucose-positron emission tomography (FDG-PET) evaluation of nodular lesions in patients with Neurofibromatosis type 1 and plexiform neurofibromas (PN) or malignant peripheral nerve sheath tumors (MPNST). Pediatr Blood Cancer. 2013 Jan;60(1):59-64.

Files

Share

How to cite

Statistics