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小儿患者的肾肿瘤及肿瘤样病变

renal tumors and tumor-like lesions in pediatric patients.

作者信息

Kissane J M, Dehner L P

机构信息

Department of Pathology, Washington University Medical Center, Barnes Hospital, St. Louis, MO 63110.

出版信息

Pediatr Nephrol. 1992 Jul;6(4):365-82. doi: 10.1007/BF00869741.

Abstract

Renal enlargement presenting as an abdominal mass(es) is attended by a lengthly differential diagnosis of non-neoplastic and neoplastic lesions with a range in serious connotations and consequences. Simple compensatory hypertrophy and unilateral multicystic dysplasia are relatively innocuous and easily recognized with appropriate imaging studies; they are also related in the sense that the normal contralateral kidney hypertrophies in the absence of a non-functioning dysplastic kidney. Bilateral nephromegaly in a neonate is generally a sign of autosomal recessive polycystic kidney disease or multicystic dysplasia secondary to distal obstructive uropathy. Primary neoplasms of kidney in the pediatric population in the past were traditionally classified as Wilms' tumors, but that erroneous practice has been eliminated with the recognition of several distinctive neoplasms in addition to classic Wilms' tumor. Separating a typical Wilms' tumor from mesoblastic nephroma, clear cell sarcoma of the kidney and the malignant rhabdoid tumor, for treatment and prognostic purposes, has become the accepted norm in the past 12-13 years. Another important advance at the cellular level is the recognition of a deletion in the short arm of chromosome 11 in the cultured cells of Wilms' tumor and in the germ cell line in certain clinical settings of Wilms' tumors. A dramatic expansion in the understanding and management of childhood renal neoplasms has occurred through the multimodality approach of laboratory investigation and applied clinical research.

摘要

以腹部肿块形式出现的肾脏增大需要对非肿瘤性和肿瘤性病变进行冗长的鉴别诊断,这些病变具有一系列严重的内涵和后果。单纯性代偿性肥大和单侧多囊性发育不良相对无害,通过适当的影像学检查很容易识别;它们在某种意义上也有关联,即正常的对侧肾脏在无功能的发育不良肾脏不存在时会发生肥大。新生儿双侧肾肿大通常是常染色体隐性多囊肾病或继发于远端梗阻性尿路病的多囊性发育不良的迹象。过去,儿科人群中的原发性肾脏肿瘤传统上被归类为威尔姆斯瘤,但随着除经典威尔姆斯瘤外还认识到几种独特的肿瘤,这种错误做法已被摒弃。在过去12至13年里,为了治疗和预后目的,将典型的威尔姆斯瘤与中胚层肾瘤、肾透明细胞肉瘤和恶性横纹肌样瘤区分开来已成为公认的规范。在细胞水平上的另一项重要进展是在威尔姆斯瘤的培养细胞以及某些威尔姆斯瘤临床情况下的生殖细胞系中识别出11号染色体短臂的缺失。通过实验室研究和应用临床研究的多模式方法,对儿童肾肿瘤的理解和管理有了显著扩展。

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