子宫内膜样腺癌合并盆腔淋巴结淋巴管肌瘤病的临床病理分析

Endometrial endometrioid adenocarcinoma with lymphangiomyomatosis of pelvis lymph node: clinicopathological analysis

目的探讨淋巴结淋巴管肌瘤病(lymphangiomyomatosis,LAM)的临床病理学特征。方法复习1例子宫内膜样腺癌合并盆腔淋巴结LAM的临床资料及病理特征,对其行HE及免疫组化En Vision法染色并复习相关文献。结果子宫内膜呈高-中分化子宫内膜样腺癌改变,盆腔淋巴结被膜及髓质内见增生的LAM细胞呈梭形、上皮样或多角形,围绕腔隙排列,细胞质嗜酸性或空泡样,细胞无明显异型性,未见核分裂象。免疫表型:SMA、Caldesmon、desmin、vimentin、ER和PR均阳性,腔隙内衬细胞CD34和D2-40均阳性,上皮样瘤细胞HMB-45阳性,Melan-A阴性,Ki-67增殖指数<1%。结论 LAM临床较为少见,好发于肺部,子宫内膜样腺癌合并盆腔淋巴结LAM极为罕见,结合组织学形态、临床特点及免疫组化染色可确诊,为今后进一步分析LAM的机制及治疗提供重要价值。

Purpose To study the clinicopathological features of lymphangiomyomatosis （ LAM ） of pelvis lymph node. Methods A patient with endometrial endometrioid adenocarcinoma and LAM was analyzed including clinical data and pathological features. HE and immunohistoehemistry of EnVision stainings were used, and the literatures were reviewed. Results Well-moderately differentiated endometrioid adenocarcinoma could be observed in the endometrium. Proloferation of LAM cells were seen in the capsule and medulla of the pelvic lymph node. The LAM cell was spindle, epitheliod and polygonal cells with oxyphilic or clear cytoplasm which arranged surrounding lacunes. The LAM cells showed no atypia and mitosis could not seen. The tumor cells showed diffusely positive for SMA, Caldesmon, desmin, vimentin, ER and PR, the cells lining the lacunes were positive for CD34 and D2-40. The epitheliod cells were positive for HMB-45 and negative for Melan-A. The Ki-67 immunostaining showed a proliferation index of 〈 1%. Conclusion LAM is an uncommon neoplastic muhisystem disease that affects the lungs mostly. Endometrial endometrioid adenocarcino-ma with LAM of pelvic lymph node is extremely rare. The diagnosis can be made according to the histological characteristics and immunohistochemical features. Moreover this conclusion will provide the clinicopathological materials for the future study about LAM.