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)

Holly Meany, Eva Dombi, James Reynolds, Millie Whatley, Ambereen Kurwa, Maria Tsokos, Wanda Salzer, Andrea Gillespie, Andrea Baldwin, Joanne Derdak, Brigitte Widemann

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Individuals with Neurofibromatosis type 1 (NF1) are at risk for developing malignant peripheral nerve sheath tumors (MPNST), which frequently arise in preexisting plexiform neurofibromas (PN). Magnetic resonance imaging (MRI) with volumetric analysis and 18-fluorodeoxyglucose-positron emission tomography (FDG-PET) were utilized to monitor symptomatic nodular lesions. Procedure: Patients with NF1 and PN on a NCI natural history trial were monitored for total body tumor volume (TTV) using volumetric MRI. FDG-PET was performed in individuals with a nodular well-demarcated lesion ≥3cm if they were growing, painful, or there was a prior history of MPNST (target lesions). Asymptomatic nodular lesions were evaluated as non-target lesions. Results: Fifteen patients (8m, 7f) median age of 18.3 years (range, 10-45 years) had a single target and non-target (n=46) nodular lesions identified on MRI. Target lesions arose within (n=8) or outside (n=3) a PN, and all but 1 had increased FDG uptake. FDG uptake was increased in non-target lesions but to a lesser degree. FDG uptake in the surrounding PN was low, similar to background activity. Pathologic evaluation performed in 11 patients demonstrated neurofibroma (n=6), atypical neurofibroma (n=2) and malignancy (n=3). Conclusions: Nodular target lesions identified on MRI in individuals with NF1 and PN demonstrate increased FDG uptake similar to MPNST, but may be benign on biopsy. Nodular target lesions may be at greater risk for malignant transformation, but their biologic and clinical behavior has not been well studied. Careful longitudinal evaluation will be required to better understand the malignant potential of these lesions.

Original languageEnglish (US)
Pages (from-to)59-64
Number of pages6
JournalPediatric Blood and Cancer
Volume60
Issue number1
DOIs
StatePublished - Jan 2013

Keywords

  • 18-fluorodeoxyglucose-positron emission tomography (FDG-PET)
  • Malignant peripheral nerve sheath tumors (MPNST)
  • Neurofibromatosis
  • Plexiform neurofibromas (PN)

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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