巨大肾素瘤的临床病理学观察

Clinical pathological observation of giant renin secreting tumor of kidney

目的阐明肾素瘤的病理形态学特征和诊断依据。方法对肾素瘤进行临床和组织病理学、免疫组织化学和超微结构观察。结果临床表现为重症肾素性高血压伴发严重心衰。镜下瘤细胞生长活跃,核异型性、核分裂像易见,瘤组织呈浸润性生长,退变坏死。免疫组化分析表明,瘤细胞Vimentin、NSE、CD34、CD68、αACT、Lysozyme、CK18、CK(Pan)、Actin(sm)呈阳性表达,Desmin、CgA、Sy、EMA、CKAEI、F8均呈阴性。超微结构观察瘤细胞胞质内可见散在或群集的圆形和菱形分泌颗粒。结论肾素瘤是一种内分泌肿瘤,一般认为来源于肾小球球旁细胞。本例免疫组化结果提示与血管内皮及神经内分泌有关,外科手术切除后症状缓解,预后良好。正确诊断有赖于光镜、免疫组化与电镜观察。

Objective To elucidate the morphologic features and dignostic evidence of renin secreting tumor of kidney.Methods The clinical features of the patient were abserved and the histopathological and ultrastructural features were studied by immunohistochemistry,light and electron microscopy.Results The patient showed severe renal hypertention and severe heart failure.Under light microscope,the tumor showed active proliferation,remarkable heterokaryosis with hyperchromatic,irregularly outlined and pleomorphic nuclei and many mitosis.The tumor cells showed invasive growth and massive necrosis.The immunohistochemical staining showed positive Vimentin,α ACT,NSE,CD34,lysozyme,CK18,CK (Pan) and actin (sm) expression and negative desmin,CgA,EMA,Sy,CK AEI and factor Ⅷ expression.Under electron microscope,many disperse and multitudinous round or diamond shaped granules were seen in the cytoplasm of the tumor cells.Conclusions The tumor is a kind of endocrine tumor and is considered to be juxtaglomerular cell origin.The immunohistochemical results suggest that the tumor is associated with neuroendocrine and vascular endothelium.The hypertension related symptoms disappeared after operation and the prognosis of the patient is good.The correct diagnosis needs the results of immunohistochemistry,light and electron microscopic observation.