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.