Resection of plexiform neurofibromas in children with neurofibromatosis type 1

BACKGROUND: Large plexiform neurofibromas can cause significant morbidity, functional impairment, and may be even life threatening. They most commonly arise in association with major nerve trunks and follow the distribution of the involved peripheral nerves, and can be subject to transformation into malignant peripheral nerve sheet tumors. They produce large infiltrative masses, which can involve major portions of the trunk or extremities. The main goal of this project is to analyze our surgical experience with plexiform neurofibromas excision and to describe our surgical technique that may help the surgeon to reduce intraoperative bleeding and facilitate tumor excision. METHODS: A retrospective chart and radiograph review was carried out for all cases of children with neurofibromatosis type 1 followed at our institution. Patients with plexiform neurofibromas of the trunk or extremities and who underwent surgical resection of the tumor were reviewed. RESULTS: Eleven female and 3 male patients with neurofibromatosis type 1 with 20 surgically treated lesions were evaluated. The mean age of the patients at time of surgery was 13.4 years (range, 2.9 to 19.1 y). The average follow-up was 65.5 months (range, 24 to 145 mo). Five lesions (25%) were surgically treated before the age of 10 years and 15 lesions (75%) were treated after the age of 10 years. Further debulking was required in 7 lesions and a below knee amputation was performed in 1 case. CONCLUSIONS: The main goal of surgical resection of large lesions is to partially restore the normal appearance and function of the affected part and to ameliorate pain, when possible. One of the major complications of this particular surgery is the potential for significant blood loss. This complication seems to be particularly frequent in large plexiform neurofibromas located in those parts of the skeleton where the application of a tourniquet is not possible. Applying large retention sutures during dissection helps in minimizing bleeding and reduces the risk of complications. LEVEL OF EVIDENCE: Level IV, retrospective study. See the guidelines online for a complete description of level of evidence.