Niu Yumeng, Xian Lei, Wang Yi, Chen Yourong, Lu Yifei, Liang Daying
Department of Cardiothoracic Surgery, The Second Affiliated Hospital of Guangxi Medical University, No.166 Da Xuedong Road Nanning Guangxi, Nanning Guangxi, 530007, China.
J Cardiothorac Surg. 2025 May 28;20(1):245. doi: 10.1186/s13019-025-03489-5.
Langerhans cell histiocytosis (LCH) is a rare disease. It mainly involves abnormal proliferation and aggregation of Langerhans cells, a type of cell of the immune system.Langerhans cytosis is more common in the bone, but it has rarely been reported in the mediastinum.
We present a case of mediastinal thymus tumor presented with Langerhans cell histiocytosis. A 40-year-old female patient presented with left chest and back pain in April 2024. Imaging revealed abnormal signal lesions on the patient's left rib and anterior superior mediastinum. The clinical diagnosis was bone destruction and mediastinal space occupation, and the postoperative pathological diagnosis was Langerhans cell histiocytosis.
Langerhans cytosis is characterized by mediastinal and thymus occupation and is relatively rare in clinical practice. For patients who tolerate surgery for a single or local lesion, surgical removal of the primary lesion should be considered. However, for patients with multiple lesions or distant metastases, clinicians should evaluate whether surgery will benefit the patient. After surgical treatment, targeted therapy or immunotherapy should be performed.
朗格汉斯细胞组织细胞增多症(LCH)是一种罕见疾病。它主要涉及免疫系统中的一种细胞——朗格汉斯细胞的异常增殖和聚集。朗格汉斯细胞增多症在骨骼中更为常见,但在纵隔中很少有报道。
我们报告一例表现为朗格汉斯细胞组织细胞增多症的纵隔胸腺肿瘤病例。一名40岁女性患者于2024年4月出现左胸和背部疼痛。影像学检查显示患者左肋骨和前上纵隔有异常信号病变。临床诊断为骨质破坏和纵隔占位,术后病理诊断为朗格汉斯细胞组织细胞增多症。
朗格汉斯细胞增多症以纵隔和胸腺占位为特征,在临床实践中相对少见。对于能够耐受手术切除单个或局部病变的患者,应考虑手术切除原发病变。然而,对于有多个病变或远处转移的患者,临床医生应评估手术是否会使患者受益。手术治疗后,应进行靶向治疗或免疫治疗。