Management of Head and Neck Plexiform Neurofibromas in Pediatric Patients With Neurofibromatosis Type 1

Contributions of inflammation and tumor microenvironment to neurofibroma tumorigenesis

“…Therefore, their associated nerves and DRGs may be more prone to injury, leading to higher neurofibroma prevalence, as nerve injury has been associated with neurofibroma development (44,45). Our observation that cervical nerves serve as a "hot zone" for plexiform neurofibroma in this mouse model appears to mimic similar observations in human NF1 studies (46,47). We hypothesize that physical motion could lead to minor but repetitive nerve injuries surrounding these vertebras compared with the nerves near other less-active spinal vertebras.…”

Insights into the Pathogenesis of NF1-Associated Neoplasms

“…Notably, they also observed preferential development of paraspinal pNFs in cervical and T5-T8 thoracic nerves, anatomic regions associated with more frequent flexion and extension and thus, more injury (Liao et al 2018). Interestingly, these "hot spots" for pNF growth observed in this mouse model are similar to that seen in human patients (Wise et al 2005, Nguyen et al 2014.…”

Massive retropharyngeal neurofibroma in a pediatric patient

“…While location of tumor within the head and neck region and massive size have been reported to worsen post operative outcomes [2,6,7] in our patient's case a total resection was feasible without compromising her speech or swallowing function and without major complications. Each case should be evaluated individually with the understanding that it is possible to completely resect a massive head and neck tumor in a pediatric patient.…”