ICNC2018 Abstracts & Symposia Proposals, ICNC 2014

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Plexiform neurofibromas in South African children with Neurofibromatosis 1
Veruschka Ramanjam

Last modified: 2014-04-03

Abstract


Plexiform Neurofibromas are serious complications of Neurofibromatosis 1 remaining a major management dilemma globally. They are both chemo and radiotherapy resistant and often difficult to excise. A viable therapeutic agent is yet to be found. 25% of children attending the multidisciplinary Neurofibromatosis clinic at our hospital presented with plexiform neurofibromas. This series aimed to better characterise this cohort to gain insight into planning future managment approaches in a resourse limited setting. Method:All children with Plexiform Neurofibromas who presented to the clinic from January 2001 to July 2013 were included, Folders were reviewed and relevant clinical details recorded. Results:23 children presented with Plexiform Neurofibromas( 9 girls, 14 boys). A family history was present in 9 of the 23. Children presented between 6 weeks and 10 years of age.(median age 3 years)MRI scans of lesions were performed on all children. 12 children had plexiform neurofibromas involving the head and neck, 3 had peri-orbital lesions,2 were facial and 1 involved occipital soft tissues. The remaining 5 children had lesions involving the lower limb(n=2), upper limb(n=1),buttock/pelvis(n=1) and lumbar paraspinal( n=1)Only 6 children underwent surgery. These will be described. Malignant transformation occured in one patient who died shortly after symptom progression.Conclusion:Managment of PLexiform Neurofibromas remains a dilemma globally. This is the first series desribing children from an african setting many of whom are from poor socio-economic environments with limited access to services.


Keywords


neurofibromatosis 1; plexiform neurofibromas

References


1. Ramanjam V et al. J Child Neurol 2006;21:63-70

2.Farner RE et al. Cancer Reseacrh 2002;62:1573-1577

3.Robertson KA et al. Lancet Oncol 2012;13:1218-1244

4.Dunn GP et al. J Neurosurg 2013;118:142-148


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