Introduction

Erdheim–Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. The clinical spectrum of ECD is diverse, varying from asymptomatic focal lesion to life-threatening multisystem infiltration. Neurological manifestations of ECD are common, mostly due to the involvement of the central nerve system. However, spinal nerve or peripheral nerve involvement has rarely been mentioned.

Case presentation

Herein, we present a case of a 32-year-old female patient complaining about radiating pain on the front and lateral side of her left thigh for 2 months. Spinal MRI with contrast enhancement showed a space-occupying lesion on the left L3/L4 intervertebral foramen, indicating an initial diagnosis of lumbar nerve schwannoma. The patient underwent surgery to remove the mass and decompress the lumbar nerve. Postoperative histological examination revealed the diffuse infiltration of foamy histiocytes that were CD68⁺, CD163⁺, and CD1a⁻ on immunostaining, which confirmed the diagnosis of Erdheim–Chester disease. The radiating pain was gradually alleviated and PET-CT was performed but showed no further involvement of ECD.

Discussion

To the best of our knowledge, this is the first case of ECD demonstrated as an infiltrative mass on the spinal nerve, with imaging manifestations and compression symptoms similar to those of peripheral nerve schwannoma.

中文翻译：

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类似于腰神经鞘瘤的埃尔德海姆-切斯特病：病例报告和文献综述。

介绍

埃尔德海姆-切斯特病 (ECD) 是一种罕见的非朗格汉斯细胞组织细胞增多症。ECD 的临床表现多种多样，从无症状的局灶性病变到危及生命的多系统浸润。ECD 的神经系统表现很常见，主要是由于中枢神经系统受累。然而，脊神经或周围神经受累却很少被提及。

案例展示

本文介绍一例32岁女性患者，主诉左大腿前侧和外侧放射痛2个月。脊柱 MRI 对比增强显示左侧 L3/L4 椎间孔有占位性病变，初步诊断为腰神经鞘瘤。患者接受了手术切除肿块并减压腰神经。^{术后组织学检查显示免疫染色显示CD68 +}、CD163 ⁺和CD1a-^的泡沫状组织细胞弥漫性浸润，证实了Erdheim-Chester病的诊断。放射痛逐渐减轻，进行了 PET-CT，但未显示 ECD 进一步受累。

讨论

据我们所知，这是首例表现为脊神经浸润性肿块的ECD病例，其影像学表现和压迫症状与周围神经神经鞘瘤相似。