University Multiprofile Hospital for Active Treatment With Emergency Medicine N. I. Pirogov, Sofia, Bulgaria.
University Hospital St. Anna, Sofia, Bulgaria.
Adv Exp Med Biol. 2023;1405:363-376. doi: 10.1007/978-3-031-23705-8_13.
The three main types of nerve sheath tumors are schwannomas, neurofibromas and perineuriomas. Multiple neurofibromas throughout the body are the hallmark of Neurofibromatosis type 1 (NF1). Spinal nerve sheath tumors are classified in the group of intradural extramedullary spinal cord tumors, in which they are the most common type (25-30%). Their incidence is 3-4 per 1 million people. Spinal schwannomas are encountered sporadically or in the context of Neurofibromatosis type 2, while neurofibromas are typical for patients with Neurofibromatosis type 1. Neurofibromas are composed predominantly of Schwann cells and fibroblasts, alongside which are also found axons, perineurial cells, mast cells and extracellular matrix. Most of the neurofibromas are asymptomatic. Any increase in the size of a neurofibroma or the presence of pain is an indicator of a possible malignant degeneration. Neurofibromas are treated surgically. Neurofibromas involve the whole nerve and cause its fusiform enlargement which makes it impossible to preserve the nerve's functions if complete tumor removal is performed. Hence, such tumors are initially observed. In case of progressive growth, the options are either resection of the tumor and immediate reconstruction with a peripheral nerve graft (e.g., nerve suralis interposition graft) or subtotal removal and follow-up. Malignant peripheral nerve sheath tumors (MPNST) are very rare tumors with incidence of around 1 per 1,000,000 people. MPNST account for 3-10% of all soft-tissue sarcomas. The most common initial symptom of MPNST is a painless mass. Any rapid increase in a subcutaneous mass or rapid onset of symptoms should raise the suspicion of a malignant tumor. In patients with diagnosed NF1, the recent rapid increase in a known lesion should raise the suspicion of malignant degeneration of the lesion and opt for active treatment. In the case of MPNST a wide surgical excision is advocated. The resectability depends greatly on the location of the tumors and varies from around 20% in paraspinal MPNST and reaches 95% in MPNST localized in the extremities. MPNST are a rare disease and should be managed by a multidisciplinary team of neurosurgeons, radiologists and oncologists.
神经鞘瘤的三种主要类型是神经鞘瘤、神经纤维瘤和神经周细胞瘤。全身多发性神经纤维瘤是神经纤维瘤病 1 型（NF1）的标志。脊髓神经鞘瘤属于硬脊膜外脊髓肿瘤组，是最常见的类型（25-30%）。其发病率为每 100 万人中有 3-4 人。脊髓神经鞘瘤偶发或见于神经纤维瘤病 2 型，而神经纤维瘤则是神经纤维瘤病 1 型患者的典型表现。神经纤维瘤主要由雪旺细胞和成纤维细胞组成，其中还存在轴突、神经周细胞、肥大细胞和细胞外基质。大多数神经纤维瘤是无症状的。神经纤维瘤的大小增加或出现疼痛是可能发生恶性变性的指标。神经纤维瘤通过手术治疗。神经纤维瘤累及整个神经并导致其梭形增大，如果完全切除肿瘤，将无法保留神经的功能。因此，通常先观察此类肿瘤。如果肿瘤生长，可选择切除肿瘤并用外周神经移植物（如腓肠神经间置移植）立即重建，或行次全切除和随访。恶性外周神经鞘瘤（MPNST）是一种非常罕见的肿瘤，发病率约为每 100 万人 1 例。MPNST 占所有软组织肉瘤的 3-10%。MPNST 的最初常见症状是无痛性肿块。任何皮下肿块的快速增大或症状的迅速出现都应引起对恶性肿瘤的怀疑。在诊断为 NF1 的患者中，已知病变的近期快速增大应怀疑病变的恶性变性，并选择积极治疗。对于 MPNST，提倡广泛的手术切除。可切除性在很大程度上取决于肿瘤的位置，从脊柱旁 MPNST 的约 20%到四肢 MPNST 的 95%不等。MPNST 是一种罕见疾病，应由神经外科医生、放射科医生和肿瘤学家组成的多学科团队进行管理。
