Analysis of clinical characteristics and surgical outcomes of neurofibromatosis in children

Abstract

Objective \nTo analyze the clinical characteristics and surgical outcomes of neurofibromatosis (NF) in children. \n \n \nMethods \nA total of 18 NF children who underwent tumor resection and were confirmed by pathological examination at Department of Neurosurgery, Xijing Hospital, Air Force Military Medical University from January 2007 to March 2019 were retrospectively analyzed. Their ages ranged from 3 to 18 years. Among them, 17 cases were NF Ⅰ and 1 was NF Ⅱ. Two cases had positive family history. Clinical manifestations at admission included milky coffee spots in 15 cases, subcutaneous nodules in 14, limb numbness in 4, visual impairment in 4, bone invasion in 2 and lumbar meningocele associated with tethered cord (tumor was identified during lysis and reconstructive operation) in 1. There were 4 cases who suffered from intracranial tumors [parafalx in 1 case, cranio-orbital communication in 2 and bilateral cerebellopontine angles (CPA) in 1] and 4 having intraspinal tumors. For patients with local compression symptoms, progressive enlargement of the tumors, or biopsy purpose, palliative tumor resection was performed and surgical outcomes were followed up. \n \n \nResults \nAmong 18 cases, early symptoms disappeared in 10 cases, were relieved in 3, and had no change in visual acuity in 4 (the same as preoperative conditions). Left hearing loss in the child with bilateral CPA tumors became severer than preoperative conditions due to left tumor resection. The follow-up duration was 2 to 143 months with an average of 74±37 months. Follow-up indicated a recurrence in 4 cases in the original surgical area (subcutaneous in 3 and CPA in 1), and a new subcutaneous lesion in 2 cases on body surface (neck in 1 and chest in 1). The 4 patients with intraspinal tumors recovered well without recurrence. Pathological results showed that Ki-67 positive cell index accounted for 1-8%. \n \n \nConclusions \nType NF Ⅰ is more common than NF type Ⅱ in children and often combined with multi-system tumors. Subcutaneous and CPA tumors tend to recur even after surgical resection. Palliative surgical decision should be made in order for the patients to maintain a satisfactory life quality. \n \n \nKey words: \nNeurofibromatoses;\xa0Child;\xa0Disease attributes;\xa0Neurosurgical procedures