朗格汉斯细胞组织细胞增生症(LCH)是一种肿瘤疾病,其特征是单核吞噬细胞系统的异常增殖,主要影响3岁以下儿童。虽然LCH可以影响几乎所有的器官,鼻窦受累是罕见的。该病例报告记录了一名9岁男孩的右侧视力障碍和间歇性头痛。CT扫描和MRI检查显示蝶窦右心房存在软组织,影响了右视神经管。活检结果证实了LCH的存在。考虑到视神经管和视力障碍的受累,我们进行了细致的清创术,随后进行了12个月的标准化疗.经过2年的随访,患者表现出明显的改善,尽管右蝶窦存在包囊性囊肿。这种情况突出了在遇到孤立的软质肿块并伴有蝶窦视力下降时考虑LCH的重要性。彻底的体检,实验室测试,和成像方法应该进行,活检是必要的,以确认病变的类型和指导适当的治疗。
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.