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伴有囊性发育异常的全叶性肾母细胞瘤病:1例经免疫组织化学研究的累及双肾的罕见病例

Panlobar nephroblastomatosis with cystic dysplasia: an unusual case with diffuse renal involvement studied by immunohistochemistry.

作者信息

Gaulier A, Boccon-Gibod L, Sabatier P, Lucas G

机构信息

Service d'Anatomie et Cytologie Pathologiques, Hôpital V. Dupouy, Argenteuil, France.

出版信息

Pediatr Pathol. 1993 Nov-Dec;13(6):741-9. doi: 10.3109/15513819309048261.

DOI:10.3109/15513819309048261
PMID:8108294
Abstract

A unilateral cystic renal process discovered prenatally was removed in a neonate. Dysplastic cysts were associated with diffuse (both intra- and perilobar) nephroblastomatosis. We describe a comprehensive immunohistological study confirming the transition observed on simple histology between the different structures: nephrogenic rests (CD9+, CD24+/-, CD56+/-), glomeruloid bodies (CD10++, CD35++), ducts lined by columnar epithelium (CD9+, CD24+, CD56++), cysts lined by cuboidal or thin epithelium (some cells CD10+, CD26+, others EMA+, CD24+). Although no typical S-shaped bodies are seen, small cysts and ducts with a columnar epithelium are considered similar. The dysplastic primitive ducts are KL1++, vimentin+/-, CD9+, CD24+. With a view to assessing dysplastic preneoplastic potential, the value of CD56 and Ki67 as activation antigens with possible prognostic significance is discussed.

摘要

一名新生儿切除了产前发现的单侧囊性肾脏病变。发育异常的囊肿与弥漫性(叶内和叶周)肾母细胞瘤病相关。我们描述了一项全面的免疫组织学研究,证实了在简单组织学上观察到的不同结构之间的转变:肾源性残留(CD9+、CD24+/-、CD56+/-)、肾小球样体(CD10++、CD35++)、柱状上皮衬里的导管(CD9+、CD24+、CD56++)、立方上皮或扁平上皮衬里的囊肿(一些细胞CD10+、CD26+,其他细胞EMA+、CD24+)。尽管未见到典型的S形小体,但具有柱状上皮的小囊肿和导管被认为相似。发育异常的原始导管KL1++、波形蛋白+/-、CD9+、CD24+。为了评估发育异常的肿瘤前潜能,讨论了CD56和Ki67作为可能具有预后意义的激活抗原的价值。

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Panlobar nephroblastomatosis with cystic dysplasia: an unusual case with diffuse renal involvement studied by immunohistochemistry.伴有囊性发育异常的全叶性肾母细胞瘤病:1例经免疫组织化学研究的累及双肾的罕见病例
Pediatr Pathol. 1993 Nov-Dec;13(6):741-9. doi: 10.3109/15513819309048261.
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[An unusual renal tumor, associating nephroblastoma, nephroblastomatosis, teratoma, and cystic dysplasia with arterial hypertension and secretion of alpha-fetoprotein].
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Diffuse hyperplastic perilobar nephroblastomatosis.弥漫性增生性叶旁肾母细胞瘤病
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Bilateral hyperplastic nephromegaly, nephroblastomatosis, and renal dysplasia in a newborn: a variety of universal nephroblastomatosis.新生儿双侧增生性肾肿大、肾母细胞瘤病和肾发育异常:一种肾母细胞瘤病的不同类型
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引用本文的文献

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Perilobar nephroblastomatosis: natural history and management.叶旁肾母细胞瘤病:自然病史与治疗
Case Rep Pediatr. 2014;2014:756819. doi: 10.1155/2014/756819. Epub 2014 Jul 9.
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Bilateral universal nephroblastomatosis in an 8-month-old infant treated with chemotherapy.一名接受化疗的8个月大婴儿的双侧普遍性肾母细胞瘤病
Pediatr Nephrol. 2005 Jul;20(7):1007-10. doi: 10.1007/s00467-005-1836-6. Epub 2005 Apr 26.