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Abstract：
OBJECTIVE: To identify the frequency of reports of sporadic schwannomatosis, the types of patients affected, and the nerves affected.
METHODS: We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria.
RESULTS: The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%).
CONCLUSIONS: This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.
METHODS: We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria.
RESULTS: The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%).
CONCLUSIONS: This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.
摘要：
目的：确定散发性神经鞘瘤病的报告频率，受影响的患者类型，和神经受到影响。
方法：我们根据既定标准确定了所有病例报告和报告散发性神经鞘瘤病患者的病例系列。
结果：最初的搜索产生了1,597项研究，其中包括15个。55人中，共有38人符合纳入标准。患者的平均年龄为48岁;41%为男性。33例患者有周围神经肿瘤,17人患有脊柱肿瘤。两个位置都有12个肿瘤。30例中有25例（83.3％）是单侧的，38例中有28例（73.7％）是节段性的（限于脊柱的一条肢体或五个或更少的连续节段）。
结论：本系统评价量化了符合散发性神经鞘瘤病标准的个体数量，并更好地描述了该人群，以促进2005年共识声明中神经纤维瘤病的分类。在处理不涉及前庭神经的多发性非皮肤神经鞘瘤时，神经肿瘤的单侧或节段分布是关键方面。
方法：我们根据既定标准确定了所有病例报告和报告散发性神经鞘瘤病患者的病例系列。
结果：最初的搜索产生了1,597项研究，其中包括15个。55人中，共有38人符合纳入标准。患者的平均年龄为48岁;41%为男性。33例患者有周围神经肿瘤,17人患有脊柱肿瘤。两个位置都有12个肿瘤。30例中有25例（83.3％）是单侧的，38例中有28例（73.7％）是节段性的（限于脊柱的一条肢体或五个或更少的连续节段）。
结论：本系统评价量化了符合散发性神经鞘瘤病标准的个体数量，并更好地描述了该人群，以促进2005年共识声明中神经纤维瘤病的分类。在处理不涉及前庭神经的多发性非皮肤神经鞘瘤时，神经肿瘤的单侧或节段分布是关键方面。
