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病例报告:正中神经巨大非典型颗粒细胞瘤

Case report: Giant atypical granular cell tumor of the median nerve.

作者信息

Liu Jun-Peng, Song Ling-Xie, Xu Zi-Yu, Wu Yue, Yao Xing-Chen, Li Meng, Du Xin-Ru

机构信息

Department of Orthopaedic Surgery and Pathology, Beijing Chaoyang Hospital, Capital Medical University, Beijing, China.

出版信息

Front Neurol. 2023 Sep 29;14:1221912. doi: 10.3389/fneur.2023.1221912. eCollection 2023.

Abstract

Granular cell tumors are extremely uncommon soft tissue neoplasms that mostly occur in the head and neck regions. Granular cell tumors are generally benign, asymptomatic, and rarely involve the median nerve. Due to the lack of awareness about granular cell tumors, they are easily misdiagnosed and mistreated in primary hospitals. Here, we report a giant atypical granular cell tumor located on the median nerve, approximately 12 cm in size, with unusual symptoms of median nerve damage. Magnetic resonance imaging revealed a fusiform mass that was hyperintense on T-weighted images and iso-hypointense on T-weighted images. The mass was subsequently biopsied and found to be a granular cell tumor. The tumor was resected, and a pathological examination was performed. Pathological examination revealed necrotic foci, abundant eosinophilic granules, pustular ovoid bodies, and multiple mitoses. Immunohistochemical staining revealed that the tumor cells were positive for S-100, CD68, SMA, SOX-10, Calretinin, and TFE3. The integrated diagnosis was an atypical granular cell tumor. To the best of our knowledge, this is the first report of an atypical granular cell tumor involving the median nerve. Furthermore, we comprehensively reviewed the existing literature to provide a concise summary of the diagnostic criteria, imaging findings, and pathological features of granular cell tumors. Given the high recurrence and metastasis rates of this disease, granular cell tumors of the median nerve should be considered when a patient presents with symptoms of median nerve impairment. The diagnosis of atypical granular cell tumors relies on pathological examination. In addition, extensive resection and long-term follow-up are necessary to improve prognosis.

摘要

颗粒细胞瘤是极其罕见的软组织肿瘤,主要发生在头颈部区域。颗粒细胞瘤通常为良性,无症状,很少累及正中神经。由于对颗粒细胞瘤认识不足,它们在基层医院很容易被误诊和误治。在此,我们报告一例位于正中神经上的巨大非典型颗粒细胞瘤,大小约12厘米,伴有正中神经损伤的异常症状。磁共振成像显示一个梭形肿块,在T加权图像上呈高信号,在T加权图像上呈等低信号。随后对该肿块进行活检,发现是颗粒细胞瘤。肿瘤被切除,并进行了病理检查。病理检查显示有坏死灶、丰富的嗜酸性颗粒、脓疱样卵圆形小体和多个核分裂象。免疫组化染色显示肿瘤细胞S-100、CD68、SMA、SOX-10、钙视网膜蛋白和TFE3呈阳性。综合诊断为非典型颗粒细胞瘤。据我们所知,这是首例累及正中神经的非典型颗粒细胞瘤报告。此外,我们全面回顾了现有文献,以简要总结颗粒细胞瘤的诊断标准、影像学表现和病理特征。鉴于该疾病的高复发和转移率,当患者出现正中神经损伤症状时,应考虑正中神经颗粒细胞瘤。非典型颗粒细胞瘤的诊断依赖于病理检查。此外,广泛切除和长期随访对于改善预后是必要的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c1e/10570615/b3f956bc3e9a/fneur-14-1221912-g0001.jpg

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