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蝶窦朗格汉斯细胞组织细胞增生症伴视力损害:病例报告及文献复习。

Langerhans cell histiocytosis in sphenoid sinus with vision impairment: Case report and literature review.

机构信息

Department of Otolaryngology-Head and Neck Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Institute of Allergy and Clinical Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

出版信息

Sci Prog. 2024 Jul-Sep;107(3):368504241257129. doi: 10.1177/00368504241257129.

Abstract

Langerhans cell histiocytosis (LCH) is a neoplastic disease characterized by aberrant proliferation of the mononuclear phagocyte system, predominantly affecting children under the age of 3 years. Although LCH can affect almost all organs, sinus involvement is rare. This case report documents a 9-year-old boy presented with vision impairment and intermittent headache on the right side. The CT scan and MRI examination revealed the presence of a soft mass in the right atrium of sphenoid sinus, which impacted the right optic canal. Biopsy results confirmed the presence of LCH. Considering the involvement of optic canal and vision impairment, meticulous debridement was performed followed by a 12-month standard chemotherapy. After 2 years of follow-up, the patient showed significant improvement, despite the presence of an encapsulated cyst in the right sphenoid sinus. This case highlights the importance of considering LCH when encountering an isolated soft mass accompanied by decreased vision in the sphenoid sinus. A thorough physical examination, laboratory tests, and imaging methods should be performed, with a biopsy being necessary to confirm the type of lesion and guide the appropriate treatment.

摘要

朗格汉斯细胞组织细胞增生症(LCH)是一种肿瘤性疾病,其特征为单核吞噬细胞系统异常增殖,主要影响 3 岁以下儿童。尽管 LCH 可影响几乎所有器官,但鼻窦受累较为罕见。本病例报告记录了 1 例 9 岁男孩,表现为视力障碍和右侧间歇性头痛。CT 扫描和 MRI 检查显示蝶窦右侧有一个软组织肿块,影响右侧视神经管。活检结果证实存在 LCH。鉴于视神经管受累和视力障碍,进行了精细清创术,随后进行了 12 个月的标准化疗。2 年后随访时,患者尽管在右侧蝶窦中有一个囊状囊肿,但仍有明显改善。本病例强调了当在蝶窦中遇到伴有视力下降的孤立性软组织肿块时,应考虑 LCH 的重要性。应进行全面的体格检查、实验室检查和影像学方法,有必要进行活检以确定病变类型并指导适当的治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4463/11363225/5c76dffd47d4/10.1177_00368504241257129-fig1.jpg

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