Gupta R K, Soni S M, Joshi V V, Cartun R, Kumar A, Jain M
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, U P India.
Indian J Cancer. 2000 Dec;37(4):184-9.
PNET of the kidney is a rare tumor with only a few published reports. In view of poorer prognosis and different therapeutic approach, renal PNET should therefore be differentiated from other primary renal neoplasma such as Wilms tumor, renal neuroblastoma and malignant rhabdoid tumor which on histology resemble renal PNET. Two cases of renal PNET have been described in this report. Cut surface of the tumor in both cases was greyish white lobulated, with multiple tiny cystic areas. Histologically, tumor consisted of loosely cohesive sheets of small to medium sized monomorphic cells with round nuclei and little cytoplasm. Tumor cells showed diffuse strong membrane positivity for MIC2 and focal weak to moderate positivity for NSE and vimentin. Renal PNET should therefore be included in differential diagnosis of rapidly enlarging renal lumps presenting with local infiltration and aggressive behaviour, particularly in children and young adults. Diffuse strong membrane positivity for MIC2 in PNET is helpful in differentiating it from other primary renal neoplasms.
肾原始神经外胚层肿瘤（PNET）是一种罕见的肿瘤，仅有少数已发表的报告。鉴于其预后较差且治疗方法不同，因此肾PNET应与其他原发性肾肿瘤如肾母细胞瘤、肾神经母细胞瘤和恶性横纹肌样瘤相鉴别，这些肿瘤在组织学上与肾PNET相似。本报告描述了2例肾PNET。两例肿瘤的切面均为灰白色分叶状，有多个微小囊变区。组织学上，肿瘤由松散聚集的中小等大小单形性细胞片组成，细胞核圆形，胞质少。肿瘤细胞MIC2呈弥漫性强膜阳性，NSE和波形蛋白呈局灶性弱阳性至中度阳性。因此，肾PNET应纳入对表现为局部浸润和侵袭性行为的快速增大的肾肿块的鉴别诊断中，特别是在儿童和年轻人中。PNET中MIC2的弥漫性强膜阳性有助于将其与其他原发性肾肿瘤区分开来。
