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

Growth behavior of plexiform neurofibromas after surgery

“…12 Surgical complications may be relatively common in NF1 patients given the frequent infiltrating and hypervascular nature of PNs; these include nerve damage, functional impairment, bleeding, and wound-healing abnormalities. [13][14][15] Prada et al 14 reported sequelae after surgery in approximately 20% of their studied patients, especially in those with head/neck tumors.…”

Plexiform Leiomyoma of the Soft Palate: A Case Study and Literature Review

“…Our case of the plexiform leiomyoma was partially well encapsulated based on pathologic analysis, however, it is noted the plexiform neurofibromas are infiltrative in nature with tumor recurrence occurring after surgery. Wise et al [6] noted that 25% of plexiform neurofibromas <5cm recurred on average after 3 years and that 100% of plexiform neurofibromas >5cm recurred on average after 3.1 years [6]. Due to the infiltrative nature these lesions may have, a study by Citak et al [23] claims interferon alpha is an alternative way to manage children with plexiform neurofibromas as radiotherapy proved deleterious secondary to the effects on bone and soft tissue in a child, surgery success being limited to tumor regrowth, and medical management being ineffective.…”

“…Morphological features of a plexiform neurofibroma with an immunoreactivity profile for desmin and smooth muscle actin produces a rare variant of a benign plexiform lesion called plexiform leiomyoma. Plexiform leiomyoma's presence has only been documented in the uterus and esophagus [1][2][3][4][5][6][7][8][9][10][11]. The following case study demonstrates the first ever finding of a plexiform leiomyoma outside the gastrointestinal tract and uterus located on the soft palate.…”

“…Plexiform neurofibroma is another plexiform tumor shown to have predilection for the head and neck region. Case reports have shown plexiform neurofibromas in areas including, but not limited to the neck, parotid gland, aretynoid, maxillary sinus, and tongue [6][7][8][9][10]. Morphological features of a plexiform neurofibroma with an immunoreactivity profile for desmin and smooth muscle actin produces a rare variant of a benign plexiform lesion called plexiform leiomyoma.…”

Airway neurofibroma

“…Plexiform neurofibromas are mostly congenital, with 50% occurring in the regions of the head, neck, face, and larynx with high rates of recurrence. 2 Their presence in the neck region can pose a risk to the airway and make intubation difficult. Though awake intubation is recommended in these situations, 3 our patient would not consent to it; consequently, we administered succinylcholine after explaining the procedure and confirming mask ventilation after induction of anesthesia.…”