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儿童转移性后肾腺瘤

Metastatic metanephric adenoma in a child.

作者信息

Renshaw A A, Freyer D R, Hammers Y A

机构信息

Department of Pathology, Baptist Hospital of Miami, FL 33176, USA.

出版信息

Am J Surg Pathol. 2000 Apr;24(4):570-4. doi: 10.1097/00000478-200004000-00012.

DOI:10.1097/00000478-200004000-00012
PMID:10757405
Abstract

Metanephric adenoma is a recently characterized renal tumor that generally occurs in adults and has an excellent prognosis. To date, only one atypical metanephric adenoma has been reported to metastasize. The authors report a case of typical metanephric adenoma that arose in the left kidney of a 7-year-old girl that was associated with metastases to the para-aortic, hilar, and aortic bifurcation lymph nodes. The tumor was 9.5 cm and was composed entirely of epithelial elements arranged in tubules, short papillae, and glomeruloid bodies with scattered psammoma bodies. No atypia and only rare mitotic activity were present. Immunohistochemically, the tumor was negative for epithelial membrane antigen, negative for keratin AE1, and focally positive for both keratin CAM5.2 and cytokeratin 7. Tumor cytogenetics revealed a normal diploid karyotype, and disomy of chromosomes 7 and 17 was confirmed by fluorescence in situ hybridization. The authors conclude that tumors with histologic, immunohistochemical, and genetic features characteristic of typical metanephric adenoma can present with metastatic disease.

摘要

后肾腺瘤是一种最近才被明确特征的肾肿瘤,通常发生于成年人,预后良好。迄今为止,仅有一例非典型后肾腺瘤被报道发生转移。作者报告了一例发生于一名7岁女孩左肾的典型后肾腺瘤病例,该病例伴有主动脉旁、肾门及主动脉分叉处淋巴结转移。肿瘤大小为9.5厘米,完全由排列成小管、短乳头及肾小球样体的上皮成分组成,并散在有砂粒体。无异型性,仅有罕见的有丝分裂活性。免疫组化显示,肿瘤上皮膜抗原阴性,角蛋白AE1阴性,角蛋白CAM5.2和细胞角蛋白7局灶阳性。肿瘤细胞遗传学显示正常二倍体核型,荧光原位杂交证实7号和17号染色体二体性。作者得出结论,具有典型后肾腺瘤组织学、免疫组化及遗传学特征的肿瘤可出现转移。

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