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目的探讨乳头状淋巴管内血管内皮细胞瘤(PILA)的临床病理学特征及诊断与鉴别诊断要点。方法分析1例脾PILA的组织病理学特点和免疫组化结果,并复习相关文献。结果肿块几乎占据整个脾,肿块中央及周边各见一处钙化区。低倍镜下肿瘤呈海绵状血管瘤样,囊状扩张的薄壁管腔内衬覆立方及靴钉样内皮细胞,并呈乳头状、肾小球样突出于腔内,核分裂罕见。免疫组化示瘤细胞CD31、CD34、D2-40、vimetin和VEGFR-3(+)、CK和S-100(-),Ki-67阳性指数<5%。结论 PILA是一种罕见的血管源性肿瘤,组织学形态及免疫组化对本病确诊具有重要作用。该瘤为低度恶性肿瘤,应与其他血管源性肿瘤鉴别。
Objective To investigate the clinical and pathological features of papillary lymphatic endothelium neoplasms (PILA) and its diagnostic and differential diagnosis. Methods One case of splenic PILA histopathological features and immunohistochemical results, and review the relevant literature. The results of the tumor almost occupy the entire spleen, the central mass and the surrounding see a calcification area. Low magnification tumor was cavernous hemangiomatous, cystic dilatation of thin-walled lined with cubic and boot-like endothelial cells, and was papillary, glomerular-like prominence in the cavity, the mitotic rare. Immunohistochemistry showed tumor cells CD31, CD34, D2-40, vimetin and VEGFR-3 (+), CK and S-100 (-), Ki-67 positive index <5%. Conclusion PILA is a rare angiogenic tumor, histological morphology and immunohistochemistry of the disease has an important role in the diagnosis. The tumor is a low-grade malignancy and should be differentiated from other vasogenic tumors.