BACKGROUND Little information about the natural history of peripheral nerve schwannomas exists in the literature. The aim of this study was to determine the natural history of those tumors both in sporadic and schwannomatosis cases to determine their growth rates and patterns. METHODS In 44 patients from 3 surgical centers, hospital charts, follow-up records, and imaging studies were reviewed. Of these patients, 7 had sporadic schwannomatosis. Histological diagnosis was obtained in 37 patients (84%). Tumor growth rates were determined by calculating the absolute and relative growth rates. RESULTS On the 47 tumors analyzed, the median tumor size at diagnosis was 1.8 cm3, and the majority of tumors were located in the lower limb (62%). The absolute growth rate ranged from - 1.13 to 23.17 cm3/year (mean, 1.69 cm3/year). Relative annual growth rates ranged from - 9 to 166%/year (mean, 33.9%/year). There was no clear correlation between initial tumor size, age at diagnosis, and tumor growth rate. Six patients (13%) harbored "fast-growing" tumors (absolute growth rate > 2 cm3/year and relative growth rate > 35%/year) while 19% of tumors demonstrate no growth or negative growth. In schwannomatosis patients, each tumor displayed a distinct growth pattern. CONCLUSION This study confirms the slow-growing nature of most, but not all, peripheral nerve schwannomas. Additional studies are mandatory to explore the environmental factors influencing growth in sporadic cases and the precise growth patterns in schwannomatosis cases to detect the rare cases of malignant transformation and pave the way to the evaluation of future clinical trials.

中文翻译：

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周围神经神经鞘瘤的自然病史。

背景技术在文献中几乎没有关于周围神经神经鞘瘤的自然病史的信息。这项研究的目的是确定散发和神经鞘瘤病病例中这些肿瘤的自然史，以确定其生长速度和模式。方法回顾了3个手术中心的44例患者的病历，医院病历，随访记录和影像学检查。这些患者中，有7例偶发性神经鞘瘤病。37例患者获得了组织学诊断（84％）。通过计算绝对和相对增长率确定肿瘤的增长率。结果在分析的47个肿瘤中，诊断时的中位肿瘤大小为1.8 cm3，大多数肿瘤位于下肢（62％）。绝对增长率为-1.13至23.17 cm3 /年（平均1.69 cm3 /年）。相对年增长率为-9至166％/年（平均33.9％/年）。初始肿瘤大小，诊断年龄和肿瘤生长率之间没有明显的相关性。6名患者（13％）患有“快速生长”的肿瘤（绝对生长率> 2 cm3 /年，相对生长率> 35％/年），而19％的肿瘤无生长或阴性生长。在神经鞘瘤病患者中，每种肿瘤均表现出不同的生长方式。结论这项研究证实了大多数（但不是全部）周围神经神经鞘瘤的缓慢生长特性。必须进行其他研究，以探索影响散发病例生长的环境因素和神经鞘瘤病病例的精确生长模式，以发现罕见的恶性转化病例，并为评估未来的临床试验铺平道路。