Abstract

BACKGROUND

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas. Their resection may lead to serious morbidity. Incidence of postoperative motor and sensory deficits is unknown, and reconstruction aimed at restoring such deficits is infrequently carried out.

OBJECTIVE

To identify the incidence and risk factors of postoperative morbidity in MPNST as well as the use and outcomes of functional reconstructions in these patients.

METHODS

Postoperative function and treatment of MPNSTs diagnosed from 1988 to 2019 in 10 cancer centers was obtained. Two models were constructed evaluating factors independently associated with postoperative motor (<M3) or critical sensory loss. Critical sensation was defined as partial or complete loss of hand, foot, or buttocks sensation.

RESULTS

A total of 756 patients (33.4% neurofibromatosis type 1, NF1) were included. MPNSTs originated in 34.4% from a major nerve. Of 658 surgically treated patients, 27.2% had <M3 muscle power and 24.3% critical sensory loss. Amputations were carried out in 61 patients. Independent risk factors for motor and sensory loss included patients with NF1, symptomatic, deep-seated, extremity, or plexus tumors originating from major nerves (all P < .05). A total of 26 patients underwent functional reconstructions. The majority (64%) of these patients regained at least M3 muscle power and 33% M4 despite 86% receiving multimodal therapy.

CONCLUSION

Resection of MPNSTs commonly results in motor and sensory deficits. Patients with NF1, symptomatic, deep-seated tumors, arising from major nerves were associated with a higher risk for developing postoperative morbidity. Functional reconstructions are infrequently performed but can improve functional outcomes.

中文翻译：

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恶性周围神经鞘瘤切除后的发病率和功能丧失

摘要

背景

恶性周围神经鞘瘤 (MPNST) 是侵袭性软组织肉瘤。它们的切除可能导致严重的并发症。术后运动和感觉缺陷的发生率尚不清楚，并且很少进行旨在恢复此类缺陷的重建。

客观的

确定 MPNST 术后并发症的发生率和危险因素，以及功能重建在这些患者中的应用和结果。

方法

获得了 1988 年至 2019 年在 10 个癌症中心诊断出的 MPNST 的术后功能和治疗。构建了两个模型来评估与术后运动 (<M3) 或临界感觉丧失独立相关的因素。临界感觉被定义为手、脚或臀部感觉的部分或完全丧失。

结果

总共包括 756 名患者（33.4% 的 1 型神经纤维瘤病，NF1）。34.4% 的 MPNST 起源于主要神经。在 658 名接受手术治疗的患者中，27.2% 的肌肉力量小于 M3，24.3% 的患者有严重的感觉丧失。61 名患者进行了截肢手术。运动和感觉丧失的独立危险因素包括 NF1、有症状、深部、四肢或起源于主要神经的神经丛肿瘤患者（所有P  < .05）。共有 26 名患者接受了功能重建。尽管 86% 的患者接受了多模式治疗，但这些患者中的大多数 (64%) 至少恢复了 M3 肌肉力量和 33% 的 M4 肌肉力量。

结论

MPNSTs 的切除通常会导致运动和感觉缺陷。由主要神经引起的 NF1、有症状、深部肿瘤的患者与发生术后并发症的风险较高有关。功能重建很少进行，但可以改善功能结果。