子宫内膜样癌合并盆腔淋巴结淋巴管肌瘤病临床病理观察

Clinicopathological features of endometrial cancer with pelvic lymph node lymphangiomyomatosis

目的探讨子宫内膜样癌合并盆腔淋巴结淋巴管肌瘤病(LAM)的临床病理学特征。方法分析1例子宫内膜样癌合并盆腔淋巴结LAM的病理诊断和鉴别诊断特征及发病机制,并结合文献进行复习。结果送检全子宫加双侧附件切除及盆腔淋巴结清扫标本,宫腔处肿瘤为高分化子宫内膜样癌,侵至宫壁肌层浅处;盆腔淋巴结13枚均未见癌转移,其中3枚淋巴结内见增生的肌样梭形细胞,梭形细胞呈网状或窦隙样排列,无明显异型性,未见病理性核分裂及坏死。免疫组化:子宫内膜样癌PAX-8、ER、PR和vimentin强(+),p16斑块状(+),WT-1、NapsinA(-);淋巴结内所见梭形细胞呈SMA、desmin和vimentin弥漫(+),HMB45局灶(+),ER、PR散在(+),MelanA(-);窦隙内衬细胞D2-40(+);Ki-67增殖指数<5%。结论子宫内膜样癌合并盆腔淋巴结LAM十分罕见,有研究认为二者存在一定的相关性,加深对该病变的认识有助于避免漏诊和误诊。

Objective To investigate the clinicopathological features of endometrial cancer with pelvic lymph node lymphangiomyomatosis.Methods The pathological diagnosis and differential diagnosis,characteristics and pathogenesis of a case of endometrial cancer with pelvic lymph node lymphangiomyomatosis were analyzed and reviewed in conjunction with review of the litrratures.Results The whole uterus plus bilateral accessory resection and pelvic lymph node dissection were sent for examination.Endometrium showed highly differentiated endometrial cancer,which invaded the superficial muscle layer of the uterine wall.Thirteen lymph nodes were detected in the pelvic cavity,and no cancer metastasis was found.Three of the lymph nodes showed proliferating myofibroblastic cells,and the spindle cells were arranged in a sinusoidal pattern.There was no obvious atypia,and no pathological mitosis and necrosis were observed.Immunohistochemically,endometrial cancer was strongly positive for PAX-8,ER,PR and Vimentin,and P16 was focally positive.WT-1 and Napsin A were negative.Spindle cells were diffusely positive for SMA,Desmin and Vimentin,HMB45 was focally positive.ER and PR were positive in some of the spindle cells.and Melan A was negative.The MIB-1(Ki-67)labeling index was less than 5%.Conclusions Endometrial cancer with pelvic lymph node LAM is very rare,and some studies find a certain correlation between the two,deepening the understanding of the disease will help to avoid missed diagnosis and misdiagnosis.