Plexiform neurofibroma
Plexiform neurofibromas can be difficult to manage especially the diffuse forms, because they tend to be infiltrative in their growth52; as such, complete surgical excision is difficult or impractical, therefore recurrence is common 3,13. One, out of the 5 patients who had surgery, in our study, developed recurrence within 5 months of surgical excision. In a longitudinal study of plexiform neurofibromas, Tucker et al observed a trend towards larger plexiform masses in children, with a higher growth rate in tumour size, than adults 3. There was no significant age or gender difference in the prevalence of plexiform neurofibromas in our study. Although 1 of our cases returned with significant growth and pain in a craniofacial plexiform neurofibroma, after 7 years’ loss to follow up, majority of our patients are unable to afford serial monitoring of plexiform neurofibromas using MRI. Plexiform neurofibromas require multidisciplinary team care due to their complexity and invasiveness. As surgical excision may be extensive, plastic reconstructive surgery may be required at the same sitting or in stages. Neurosurgery may be required to intervene for orbital plexiform neurofibromas, which invade the intracranial cavity, as was the case in almost half of our cranio-facial neurofibromas. In one case, the neurofibroma invaded the region of the facial nerve, causing facioparesis. This case should benefit from the expertise of a maxilla-facial surgeon in a multidisciplinary team setting without additional logistic delays such as scheduling fresh outpatient consultations, in a different clinic or additional cost. In our study, there was a tendency for patients to fail to return for follow-up (becoming lost to follow up) when they were referred to other specialty surgical clinics for review or additional consultation. It is possible that the additional cost of including an additional surgical team, including the logistics and delays, that may occur with scheduling joint surgeries, may have discouraged them. This would be avoided if a multidisciplinary team were already established and working seamlessly as a single unit sharing information and resources. Surgical excision remains the main form of management available to our patients as recent therapies such as interferon alpha-2b and the tyrosine kinase inhibitor, imatinib are not accessible13.