Abstract
BACKGROUND: 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. CASE PRESENTATION: 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. CONCLUSIONS: 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.