Halliday A L, Sobel R A, Martuza R L
Department of Surgery (Neurosurgery Service), Massachusetts General Hospital, Boston.
J Neurosurg. 1991 Feb;74(2):248-53. doi: 10.3171/jns.1991.74.2.0248.
Benign spinal nerve sheath tumors (neurofibromas and schwannomas) often occur on dorsal nerve roots sporadically or in neurofibromatosis types 1 and 2. These are histologically benign tumors, and distinction between them is frequently not made by clinicians. To determine if there is a correlation between the histological pattern of benign spinal nerve sheath tumors and the type of neurofibromatosis, the clinical and pathological features of these tumors (86 surgical specimens and five autopsies) in 68 patients were reviewed. The patients were classified into one of four categories: neurofibromatosis type 1, neurofibromatosis type 2, uncertain, or sporadic. The diagnostic criteria used for neurofibromatosis types 1 and 2 were established by the National Institutes of Health. Patients who did not fulfill criteria for either neurofibromatosis type 1 or 2 but who had multiple nervous system tumors or other stigmata of neurofibromatosis were designated "uncertain." Spinal nerve sheath tumors were considered sporadic in 42 cases (40 schwannomas and two neurofibromas). In the 14 patients with neurofibromatosis type 1, all spinal nerve sheath tumors were neurofibromas. In six of the seven patients with neurofibromatosis type 2, all spinal nerve sheath tumors were schwannomas. One patient with neurofibromatosis type 2 had a spinal nerve sheath schwannoma and a tumor with features of both tumor types. The authors conclude that spinal nerve sheath tumors in patients with neurofibromatosis type 1 are neurofibromas. In contrast, spinal nerve sheath tumors occurring in neurofibromatosis type 2 or sporadically are most frequently schwannomas. The distinct histological features of these tumors may reflect different pathogenetic mechanisms even though they arise at identical sites in neurofibromatosis types 1 and 2.
良性脊神经鞘瘤（神经纤维瘤和施万细胞瘤）常散发于背神经根，或见于1型和2型神经纤维瘤病患者。这些肿瘤在组织学上为良性，临床医生常常无法区分它们。为了确定良性脊神经鞘瘤的组织学模式与神经纤维瘤病类型之间是否存在关联，我们回顾了68例患者中这些肿瘤（86份手术标本和5份尸检标本）的临床和病理特征。患者被分为四类之一：1型神经纤维瘤病、2型神经纤维瘤病、不确定或散发型。1型和2型神经纤维瘤病的诊断标准由美国国立卫生研究院制定。未符合1型或2型神经纤维瘤病标准，但有多发性神经系统肿瘤或其他神经纤维瘤病体征的患者被归为“不确定”。42例脊神经鞘瘤被认为是散发型（40例施万细胞瘤和2例神经纤维瘤）。在14例1型神经纤维瘤病患者中，所有脊神经鞘瘤均为神经纤维瘤。在7例2型神经纤维瘤病患者中的6例中，所有脊神经鞘瘤均为施万细胞瘤。1例2型神经纤维瘤病患者有1个脊神经鞘施万细胞瘤和1个具有两种肿瘤类型特征的肿瘤。作者得出结论，1型神经纤维瘤病患者的脊神经鞘瘤为神经纤维瘤。相比之下，2型神经纤维瘤病患者或散发型患者中出现的脊神经鞘瘤最常见的是施万细胞瘤。这些肿瘤独特的组织学特征可能反映了不同的发病机制，尽管它们在1型和2型神经纤维瘤病的相同部位发生。
