本帖最后由 pathology 于 2014-9-5 11:24 编辑
本周病例（2014.8.11—2014.8.17）
1、你考虑什么诊断？
先天性脊柱裂合并脊髓脊膜膨出伴***性残留（Congenital spina bifida and myelomeningocele with nephrogenic rests）
VG染色
EMA
GFAP
Benign ectopic immature renal tissue is a rare finding, and the combination of immature or dysplastic renal tissue and hamartomas or choristomas of the lumbosacral region has been documented only a few times. One of these cases was associated with spina bifida, and one with myelomeningocele. None was associated with malignant behavior. However, cases of Wilms' tumor that arise in the lumbosacral region in association with spina bifida and pre-existent masses have been described.
The presence of nephrogenic rests in a lipomyelomeningocele might be explained if neural tube defects interfere with the migration of renal tissue. In support of this is the frequent association of neural tube defects with renal anomalies. Alternatively, aberrant induction of renal tissue might explain this lesion. Normal development of the nephron is induced in metanephric blastema by the ureteric bud; however, central nervous system tissue is capable of inducing nephron formation in cultured tissue taken from undifferentiated Wilms' tumor, and of inducing tubules in metanephric mesenchyme. The expression of nerve growth factor receptor in developing nephrogenic tissue is required for the formation of the kidney tubules.
2、需要做什么鉴别诊断？
畸胎瘤、脂肪瘤伴有异源性成分、错构瘤/迷离瘤等
3、本病预后如何？
本病为良性，单纯切除后预后良好，目前尚无***性残留成分恶变的病例报告。
－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－－
本期病例比较少见但非常典型，考虑到脊膜膨出的临床病史，结合组织学形态，辅以免疫组化不难诊断。综合回答思路，派瑞松加一分，xhyong累积半分！
希望大家一如既往的支持病理每周一题， | 
发表于 2014-8-12 13:26
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发表于 2014-8-12 22:34
