摘要目的:探讨儿童分枝杆菌梭形细胞假瘤的临床病理特征、诊断及鉴别诊断，以提高对该罕见疾病的认识。方法:对2例分枝杆菌梭形细胞假瘤的临床资料、病理组织学特征、免疫表型及特殊染色进行分析，并复习相关文献。结果:2例患儿均为男性，就诊年龄分别为11个月及1岁10个月，但发病年龄均在4个月左右，慢性病程，发病部位分别为淋巴结及皮肤，第1例患儿伴间断发热4个月余，第2例患儿不伴发热，均在外院接受抗感染治疗但无明显效果，于外院行肿块切除术，病理组织学形态均为大量增生的梭形细胞，伴背景炎性细胞浸润，增生梭形细胞形态温和，无明显异型，未见核分裂象，核呈泡状，部分见细小核仁，胞质丰富、粉染。免疫组织化学染色显示增生的梭形细胞呈CD68弥漫强阳性表达，Ki-67阳性指数低。抗酸染色在增生的梭形细胞胞质内见大量抗酸杆菌。第1例患儿抗结核治疗过程中肝门淋巴结进行性肿大，继而出现梗阻性黄疸，且存在全身播散病变而放弃治疗，并失访；第2例患儿术后10 d原发部位复发，且逐渐出现其他部位皮肤结节和浅表淋巴结肿大，皮肤组织活检病原微生物高通量基因检测显示结核分枝杆菌复合群，给予联合抗结核治疗11 d后，病情明显好转。结论:儿童分枝杆菌梭形细胞假瘤是非常罕见的良性病变，以分枝杆菌感染导致梭形组织细胞增生为特点，抗酸染色找到大量抗酸杆菌是确诊方法。

abstractsObjective:To study the clinicopathologic features, and the diagnosis and differential diagnosis of mycobacterial spindle cell pseudotumor in children.Methods:The clinical data, histopathological features, immunophenotype and special staining of 2 mycobacterial spindle cell pseudotumors were analyzed. The related literatures were reviewed.Results:The age of the two boys was 11 months and 22 months respectively, but their clinical symptoms became apparent at the age of about 4 months. The lesions involved lymph nodes and skin. The first patient also had fever for more than 4 months. Both patients received anti-inflammatory treatment in the outside hospital, but had no obvious improvements of the symptoms. A tumor resection was performed at the outside hospital. Histologically, mycobacterial spindle cell pseudotumor consisted of bland spindle cells, which formed fascicles, without any obvious atypia and mitoses. The cell nuclei were vesicular, with small nucleoli and abundant cytoplasm in some of the cases. The spindle cells expressed histiocyte-associated markers, such as CD68. The Ki-67 proliferation index was low. The mycobacteria were usually readily highlighted by acid-fast staining, which located in the cytoplasm of proliferative spindle cells. In the first case, there was obstructive jaundice because of the progressive enlargement of live portal lymph nodes and systemic disseminated lesions. The second patient had disease recurrence after only operation, and gradually developed other skin nodules and superficial lymph node enlargement. The high-throughput molecular analysis of the skin biopsy confirmed the diagnosis of mycobacterium tuberculosis. After 11 days of anti-tuberculosis treatment, the patient′s condition improved significantly.Conclusions:Mycobacterial spindle cell pseudotumor in children is a very rare benign lesion. It is characterized by spindle-histiocyte proliferation caused by mycobacterium infection. An acid-fast stain appears necessary for confirming the diagnosis.