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伴有淋巴浸润的肿瘤样结节病样反应,显示从左侧颈静脉孔向颅内延伸:病例说明

Tumor-mimicking sarcoid-like reaction with lymphoid infiltration showing intracranial extension from the left jugular foramen: illustrative case.

作者信息

Kim Young Ju, Nishihara Masamitsu, Iwahashi Hirofumi, Yamanishi Shunsuke, Ashida Noriaki, Hosoda Kohkichi, Nagashima Hiroaki, Tanaka Kazuhiro, Sasayama Takashi

机构信息

Department of Neurosurgery, Nishi-Kobe Medical Center, Kobe, Hyogo, Japan.

Department of Neurosurgery, Kobe University Graduate School of Medicine, Kobe, Hyogo, Japan.

出版信息

J Neurosurg Case Lessons. 2025 Jul 28;10(4). doi: 10.3171/CASE25316.

Abstract

BACKGROUND

Accumulation of normal lymphocytes within the intradural extramedullary space is rare. To the best of the authors' knowledge, no prior reports have described a sarcoid-like reaction with lymphoid infiltration arising around the jugular foramen and adhering to cranial nerves. Here, the authors present the case of a tumor-mimicking sarcoid-like reaction with intracranial extension from the left jugular foramen, adherent to the cerebellum, medulla oblongata, and lower cranial nerves.

OBSERVATIONS

A 64-year-old woman with a history of serous otitis media and presumed tuberculous meningitis presented with persistent headache, left-sided tinnitus, hearing loss, hoarseness, and progressive vertigo. Imaging revealed a 2-cm enhancing mass around the left jugular foramen, extending intracranially and adhering to the cerebellum and medulla oblongata. The preoperative differential diagnoses included schwannoma, glomus tumor, meningioma, lymphoma, and granuloma. A retrosigmoid approach was used for biopsy and partial resection. Intraoperative pathology revealed lymphocytic infiltration without malignancy. Definitive pathology confirmed sarcoid-like lymphoid infiltration without evidence of neoplastic or infectious etiology. The patient showed a remarkable response to corticosteroid therapy, with both clinical and radiographic improvement and no recurrence at 20 months.

LESSONS

Recognition of this rare entity and intraoperative identification of lymphocytic infiltration may support the use of corticosteroids and influence surgical decision-making. https://thejns.org/doi/10.3171/CASE25316.

摘要

背景

正常淋巴细胞在硬脊膜外髓外间隙积聚的情况罕见。据作者所知,此前尚无报告描述过在颈静脉孔周围出现类似结节病反应并伴有淋巴样浸润且累及脑神经的情况。在此,作者报告一例肿瘤样的类似结节病反应,起源于左侧颈静脉孔并向颅内扩展,累及小脑、延髓和下组脑神经。

观察结果

一名64岁女性,有浆液性中耳炎病史且疑似患有结核性脑膜炎,出现持续性头痛、左侧耳鸣、听力丧失、声音嘶哑和进行性眩晕。影像学检查显示左侧颈静脉孔周围有一个2厘米大小的强化肿块,向颅内扩展并累及小脑和延髓。术前鉴别诊断包括神经鞘瘤、球瘤、脑膜瘤、淋巴瘤和肉芽肿。采用乙状窦后入路进行活检和部分切除术。术中病理显示淋巴细胞浸润但无恶性肿瘤。最终病理证实为类似结节病的淋巴样浸润,无肿瘤性或感染性病因证据。患者对皮质类固醇治疗反应显著,临床和影像学均有改善,20个月时无复发。

经验教训

认识到这种罕见疾病并在术中识别淋巴细胞浸润可能有助于皮质类固醇的使用并影响手术决策。https://thejns.org/doi/10.3171/CASE25316

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8a00/12305358/b397a7b52dd8/CASE25316_figure_1.jpg

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