Division of Paediatric Neurosurgery, Department of Neurosurgery, University Hospital of Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany.
Department of Neurosurgery, University Hospital Tübingen, Tübingen, Germany.
Childs Nerv Syst. 2020 Oct;36(10):2433-2442. doi: 10.1007/s00381-020-04703-6. Epub 2020 Jun 6.
Peripheral nerve sheath tumours in children are a rare and heterogeneous group, consisting mostly of benign tumours as well as malignant neoplasms. Especially in the paediatric population, diagnostics and indication for therapy pose relevant challenges for neurosurgeons and paediatric neurologists alike. Most paediatric cases that need surgical intervention are associated to neurofibromatosis type 1 (NF1).
We retrospectively reviewed all paediatric cases treated at the Department of Neurosurgery in Tübingen between 2006 and 2017 for peripheral nerve sheath tumours. We analysed clinical signs, symptoms, histology, association to an underlying phacomatosis and sensory/motor function.
Of the 82 identified patients, the majority had NF1 (76.8%). Nine children bore a sporadic tumour without underlying phacomatosis (11%), 8 had NF2 (9.8%) and 2 schwannomatosis (2.4%), A total of 168 surgical interventions were performed, and 206 tumours were removed. Indication for surgery was in most instances significant tumour growth (45.2%) followed by pain (33.9%). New deficits led to surgery in 12.5% of interventions; malignancy was suspected in 8.3%. Histopathology revealed mostly neurofibromas (82.5%), divided into cutaneous neurofibromas (10.7%), infiltrating plexiform neurofibromas (25.7%) and peripheral nerve-born neurofibromas (46.1%). 12.1% of tumours were schwannomas, 2.9% MPNST, 1.5% ganglioneuroma (n = 3) and 1 hybrid-neurofibroma and perineurinoma each. Leading symptoms, such as pain and motor and sensory deficits, improved after 125/166 interventions (74.4%), remained unchanged following 39 interventions (23.2%) and worsened in 4 occasions (2.4%).
Surgery is safe and effective for (neurofibromatosis associated) peripheral nerve sheath tumours in the paediatric population; however, management needs a multidisciplinary setting. We propose early surgical resection in paediatric patients with peripheral nerve sheath tumours with significant growth, or pain, or motor deficit, or suspected malignancy.
儿童外周神经鞘肿瘤是一种罕见且异质性的肿瘤，主要由良性肿瘤和恶性肿瘤组成。特别是在儿科人群中，神经外科医生和儿科神经科医生都面临着诊断和治疗适应证方面的挑战。大多数需要手术干预的儿科病例都与神经纤维瘤病 1 型（NF1）有关。
我们回顾性分析了 2006 年至 2017 年期间在图宾根神经外科接受治疗的所有儿童外周神经鞘肿瘤病例。我们分析了临床症状、体征、组织病理学、与潜在神经皮肤瘤的关系以及感觉/运动功能。
在 82 例确诊的患者中，大多数为 NF1（76.8%）。9 例患儿为无潜在神经皮肤瘤的散发性肿瘤（11%），8 例为 NF2（9.8%），2 例 schwannomatosis（2.4%）。共进行了 168 次手术干预，切除了 206 个肿瘤。手术的指征主要是肿瘤的显著生长（45.2%），其次是疼痛（33.9%）。12.5%的手术是由于新的功能障碍，8.3%怀疑为恶性肿瘤。组织病理学显示主要为神经纤维瘤（82.5%），分为皮肤神经纤维瘤（10.7%）、浸润性丛状神经纤维瘤（25.7%）和外周神经源性神经纤维瘤（46.1%）。12.1%的肿瘤为 schwannoma，2.9%为 MPNST，1.5%为 ganglioneuroma（n=3），各有 1 例为混合性神经纤维瘤和 perineurinoma。主要症状如疼痛、运动和感觉功能障碍，在 125/166 次干预后得到改善（74.4%），39 次干预后保持不变（23.2%），4 次恶化（2.4%）。
手术治疗儿童外周神经鞘肿瘤（包括神经纤维瘤病相关肿瘤）是安全有效的，但需要多学科管理。我们建议对有显著生长、疼痛、运动障碍或怀疑恶性肿瘤的儿童外周神经鞘肿瘤患者进行早期手术切除。
