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目的探讨合并副瘤综合征肾上皮样血管平滑肌脂肪瘤(EAML)的临床病理特征。方法对合并有发热、贫血、血沉增快、血小板增多、肝功能异常副瘤综合征的右肾占位患者,行腹腔镜下右肾根治性切除术。病理提示低度恶性EAML。通过临床及病理资料并进行随访,结合复习文献,对EAML及肾肿瘤副瘤综合征临床病理特点、生物学行为及预后进行分析。结果病理检查:肿瘤包膜完整,来源于右肾中下极;切面为鱼肉样组织,伴灶性坏死组织;镜下:密集分布的上皮样细胞,无脂肪组织;免疫组化染色黑色素瘤相关抗原(CD34、SMA、Melan-A)阳性、上皮膜抗原(EMA)阴性。病理报告:低度恶性右肾EAML,输尿管残端、血管残端及肾周组织未见肿瘤浸润。术后随访肿瘤未见复发转移。结论 EAML是一种罕见的、有恶性潜能的间叶肿瘤,有一定的病理特点及生物学行为。合并副瘤综合征罕见,症状消失可能与预后有关。
Objective To investigate the clinicopathological features of renal cell carcinoma of the renal epithelium with angioelastoma (EAML). Methods The patients with right renal insufficiency complicated with fever, anemia, erythrocyte sedimentation rate, thrombocytosis and abnormal liver function syndrome were treated by laparoscopic radical nephrectomy. Pathology suggests low-grade EAML. Through clinical and pathological data and follow-up, combined with the review of literature, EAML and renal tumor paratumor syndromes, the clinicopathological features, biological behavior and prognosis were analyzed. Results Pathological examination: The tumor capsule was intact, originated from the middle and lower polar of the right kidney. The cut surface was fish-like tissue with focal necrosis tissue. Microscope: densely distributed epithelioid cells without adipose tissue; immunohistochemical staining of melanoma Antigen (CD34, SMA, Melan-A) positive, epithelial membrane antigen (EMA) negative. Pathological report: low grade malignant right kidney EAML, ureteral stump, vascular stump and perirenal tissue tumor infiltration. No follow-up of tumor recurrence and metastasis. Conclusion EAML is a rare malignant mesenchymal tumor with certain pathological features and biological behavior. Meridan tumor syndrome rare, the disappearance of symptoms may be related to the prognosis.