Department of Neurosurgery, Bâtiment Babinski, Groupe Hospitalier Pitié-Salpêtrière, APHP, Sorbonne Universités, 47-83 boulevard de l'Hôpital, 75013, Paris, France.
Genetics and Development of Brain Tumors, CRICM INSERM U1127 CNRS UMR 7225, Hôpital de la Pitié-Salpêtrière, Brain Institute, Paris, France.
Neurosurg Rev. 2023 Oct 19;46(1):275. doi: 10.1007/s10143-023-02186-y.
Pelvic schwannomas are rare tumors that may occur either sporadically or in the context of schwannomatosis. We retrospectively reviewed the charts of patients harboring a pelvic schwannoma under conservative management or operated at our reference center between 2016 and 2023. All patients were operated by a multidisciplinary team, combining a vascular surgeon and a neurosurgeon. Twenty-four patients harboring 33 pelvic tumors were included in the cohort, including 12 patients with sporadic lesions, 2 patients with NF2-related schwannomatosis, and 10 patients with NF2-independent schwannomatosis. Multi-nodular tumors were more frequent in schwannomatosis compared to sporadic cases (p = 0.005). The mean age at diagnosis was 41 years old. Schwannomas were located on branches of the sciatic nerve (23/33, 70%), the femoral nerve (6/33, 18%), and the obturator nerve (4/33, 12%). Over the course of the study, 16 patients were operated, including 11 sporadic cases. The indication for surgery was pain (12/16, 75%) or tumor growth (4/16, 25%). Complete resection was achieved in 14 of 16 patients (87%). The mean post-operative follow-up was 37 months (range: 2-168 months). At last-follow-up, complete pain relief was achieved in all 12 patients with pre-operative pain. Post-operative morbidity included 3 long-term localized numbness and one MRC class 4 motor deficit in a multi-nodular tumor in a schwannomatosis patient. Despite its limited size, our series suggests that nerve-sparing resection of pelvic schwannomas offers satisfying rates of functional outcome both in sporadic and schwannomatosis cases, except for multi-nodular tumors.
盆腔神经鞘瘤是一种罕见的肿瘤，可散发发生，也可发生于神经鞘瘤病。我们回顾性分析了 2016 年至 2023 年间在我们的中心接受保守治疗或手术治疗的盆腔神经鞘瘤患者的病历。所有患者均由一个多学科团队进行手术，其中包括血管外科医生和神经外科医生。该队列共纳入 24 例 33 个盆腔肿瘤患者，包括 12 例散发病变患者、2 例 NF2 相关神经鞘瘤病患者和 10 例 NF2 不相关神经鞘瘤病患者。神经鞘瘤病患者的多结节肿瘤比散发患者更为常见（p=0.005）。诊断时的平均年龄为 41 岁。神经鞘瘤位于坐骨神经分支（23/33，70%）、股神经（6/33，18%）和闭孔神经（4/33，12%）。在研究过程中，有 16 例患者接受了手术，其中 11 例为散发病变。手术指征为疼痛（12/16，75%）或肿瘤生长（4/16，25%）。16 例患者中有 14 例（87%）实现了完全切除。术后平均随访时间为 37 个月（范围：2-168 个月）。末次随访时，所有术前有疼痛的 12 例患者均完全缓解疼痛。术后并发症包括 3 例长期局部麻木和 1 例多结节肿瘤的神经鞘瘤病患者出现 MRC 分级 4 级运动障碍。尽管我们的研究规模较小，但我们的研究结果表明，对于散发和神经鞘瘤病患者，除多结节肿瘤外，保留神经的盆腔神经鞘瘤切除术均能获得令人满意的功能结果。
