Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA.
Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA.
World Neurosurg. 2020 Sep;141:e670-e676. doi: 10.1016/j.wneu.2020.05.280. Epub 2020 Jun 6.
Intraneural (IN) perineuriomas are a rare benign hypertrophic nerve tumor, most frequently occurring in young patients. Patients with IN perineurioma have been anecdotally found to have limb undergrowth; however, this has not been systematically evaluated.
Archived electronic records from 1990 to 2018 from a single institution were reviewed for pathology or radiology reports documenting a diagnosis of IN perineurioma. This identified 111 patients; 3 patients with IN perineurioma of cranial nerves were excluded. We further reviewed the 108 patients and identified those with a documented limb length discrepancy (LLD) or hand/foot size discrepancy (HFD) and tried to correlate findings with nerve-territory distribution.
Twenty-seven (25.0%) patients had either LLD or HFD. Nine patients had only an LLD, 6 patients had only an HFD, and 12 patients had both. Patients with undergrowth were significantly younger at diagnosis than patients without (6.14 vs. 22.9 years, respectively). Although there was a trend toward a greater incidence of LLD in lower extremity IN perineuriomas, this was not statistically significant. Patients with proximal IN perineuriomas had a higher incidence of LLD or HFD than patients with distal IN perineuriomas. The difference between the 2 groups was statistically significant (P < 0.0001). All instances of undergrowth were explained by nerve-territory bone innervation.
Limb undergrowth occurs in the affected nerve territory and is likely under-reported in patients with IN perineuriomas. Within our series, patients with documented LLD and HFD were likely to be significantly younger at diagnosis than patients without undergrowth.
神经内（IN）周围神经鞘瘤是一种罕见的良性肥大性神经肿瘤，多见于年轻患者。有报道称 IN 周围神经鞘瘤患者存在肢体生长迟缓，但尚未对此进行系统评估。
回顾单中心 1990 年至 2018 年的存档电子病历，寻找病理或放射学报告中记录的 IN 周围神经鞘瘤诊断。共确定了 111 例患者，排除了 3 例发生于颅神经的 IN 周围神经鞘瘤患者。我们进一步对这 108 例患者进行了评估，确定了有记录的肢体长度差异（LLD）或手足大小差异（HFD）的患者，并尝试将这些发现与神经支配区域相关联。
27 例（25.0%）患者存在 LLD 或 HFD。9 例患者仅存在 LLD，6 例患者仅存在 HFD，12 例患者同时存在两者。生长迟缓的患者诊断时明显比无生长迟缓的患者年轻（分别为 6.14 岁和 22.9 岁）。尽管下肢 IN 周围神经鞘瘤中 LLD 的发生率有增高趋势，但差异无统计学意义。与远端 IN 周围神经鞘瘤相比，近端 IN 周围神经鞘瘤患者的 LLD 或 HFD 发生率更高，差异具有统计学意义（P < 0.0001）。所有生长迟缓的病例均归因于神经支配区域的骨骼神经支配。
生长迟缓发生于受累神经支配区域，在 IN 周围神经鞘瘤患者中可能报告不足。在我们的系列中，有记录的 LLD 和 HFD 的患者比无生长迟缓的患者更可能在诊断时明显更年轻。
