Hand and Upper Extremity Surgery Unit, European Hospital Georges Pompidou, Assistance publique-Hôpitaux de Paris, 20, rue Leblanc, 75908, Paris Cedex 15, France.
Faculté de Pharmacie de Paris, Université Paris Descartes-Sorbonne, Paris Cité, Paris, France.
Acta Neurochir (Wien). 2020 Aug;162(8):1883-1889. doi: 10.1007/s00701-020-04430-6. Epub 2020 Jun 16.
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.
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.
On the 47 tumors analyzed, the median tumor size at diagnosis was 1.8 cm, and the majority of tumors were located in the lower limb (62%). The absolute growth rate ranged from - 1.13 to 23.17 cm/year (mean, 1.69 cm/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 cm/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.
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.
关于周围神经鞘瘤的自然病史，文献中资料甚少。本研究旨在确定散发性和神经鞘瘤病患者中这些肿瘤的自然病史，以确定其生长速度和模式。
在 3 个外科中心的 44 名患者中，对病历、随访记录和影像学研究进行了回顾。其中 7 例为散发性神经鞘瘤病。在 37 名患者（84%）中获得了组织学诊断。通过计算绝对和相对增长率来确定肿瘤的生长速度。
在分析的 47 个肿瘤中，诊断时的中位肿瘤大小为 1.8 厘米，大多数肿瘤位于下肢（62%）。绝对生长率范围为-1.13 至 23.17 厘米/年（平均 1.69 厘米/年）。相对年增长率范围为-9 至 166%/年（平均 33.9%/年）。初始肿瘤大小、诊断时的年龄与肿瘤生长速度之间无明显相关性。6 名患者（13%）存在“快速生长”的肿瘤（绝对生长率>2 厘米/年，相对生长率>35%/年），而 19%的肿瘤无生长或负生长。在神经鞘瘤病患者中，每个肿瘤都表现出不同的生长模式。
本研究证实了大多数（但不是全部）周围神经鞘瘤的生长缓慢。需要进一步的研究来探讨影响散发性病例生长的环境因素，以及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，并为未来临床试验的评估铺平道路。
