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目的探讨淋巴管肌瘤病(LAM)的临床病理特征、鉴别诊断、治疗与预后。方法对1例子宫淋巴管肌瘤病进行临床病理特征及免疫组化分析,并复习相关文献。结果镜下见瘤细胞形成多量囊腔样、网状或窦样不规则腔隙,增生的瘤细胞呈梭形、多角形或上皮样,围绕腔隙排列,细胞质透亮或嗜酸性,细胞异型不明显,核分裂少见。卵巢病灶见瘤组织侵犯神经纤维,淋巴结病灶脉管内见瘤栓。检出淋巴结数10枚,均可见瘤细胞。免疫组化:SMA、actin、caldesmon、desmin、ER和PR均(+),上皮样瘤细胞HMB45(+),Ki-67增殖活性<1%。结论子宫LAM罕见,易误诊为其他疾病,LAM病理形态上为良性,但具有一定的侵袭性,可以侵犯神经和在淋巴管内形成瘤栓,能沿淋巴管系统播散。
Objective To investigate the clinical and pathological features, differential diagnosis, treatment and prognosis of lymphangiomyosarcoma (LAM). Methods One case of uterine lymphangiomyoma was analyzed with clinicopathological features and immunohistochemistry, and the related literatures were reviewed. Results Microscopically, tumor cells were found to form a large number of cystoid, reticular or sinusoids with irregular cavities. The hyperplastic tumor cells were spindle, polygonal or epithelial, arranged around the cavity with cytoplasm translucent or eosinophilic. Obviously, mitotic rare. Ovarian lesions see tumor tissue invasion of nerve fibers, lymph node lesions see tumor suppository. Detected the number of lymph nodes 10, are visible tumor cells. Immunohistochemistry: SMA, actin, caldesmon, desmin, ER and PR (+), epithelial tumor cells HMB45 (+), Ki-67 proliferation activity <1%. Conclusions Uterine LAM is rare and easily misdiagnosed as other diseases. LAM is benign, but it has certain aggressiveness. It can infiltrate nerves and form tumor suppositories in the lymphatic vessels, which can spread along the lymphatic system.