Management of head and neck plexiform neurofibromas in pediatric patients with neurofibromatosis type 1

Objectives: To identify presenting symptoms, growth patterns, and outcomes of head and neck plexiform neurofibromas (PNs) in children with neurofibromatosis type 1 (NF-1); to determine which patients may benefit most from operative intervention in terms of duration of disease-free progression, perioperative morbidity, identification of malignancy, and symptom relief. Design: A retrospective review of 39 pediatric patients with NF-1 who had PNs of the head and neck managed at a single tertiary referral center. Results: Thirty-nine patients had 49 head and neck PNs, 11 small (<= 5 cm) and 38 massive (> 5 cm and/or involving multiple deep neck sites). Thirty-nine surgical procedures were performed on 18 of 35 patients with massive disease, and 4 procedures were performed on 4 of 11 patients with small tumors. Tumors recurred in 1 (25%) of 4 patients with small tumors and in IS (100%) of 18 patients with massive tumors (P=.001; mean time to regrowth, 3.1 years.) Conclusions: Size and location of PN tumors most influenced presentation of clinical symptoms. Complete tumor resection was possible only in patients with small PNs. Patients with PNs of the head and neck were more likely to benefit from surgery if the indications were to (1) exclude malignancy in a rapidly enlarging mass-, (2) provide relief from neurogenic pain or motor weakness; (3) improve symptoms caused by airway compression; or (4) enhance cosmesis in those with disfiguring disease.