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成熟型囊性腹膜后畸胎瘤伴分化良好的肾组织成分:与脊柱裂的关系

Mature cystic retroperitoneal teratoma with well differentiated renal elements: relation to spinal dysraphism.

作者信息

Sinha Anand, Sabharwal Arvind, Yadav Nandini, Gupta R K, Patir Rana

机构信息

Department of Pediatric Surgery, Fortis Memorial Research Institute, Gurgaon, Haryana, India.

Department of Pathology, Fortis Memorial Research Institute, Gurgaon, Haryana, India.

出版信息

European J Pediatr Surg Rep. 2014 Jun;2(1):46-9. doi: 10.1055/s-0033-1351393. Epub 2013 Aug 5.

Abstract

Retroperitoneum is a relatively uncommon site for pediatric teratomas. Rarely, such tumors can have an intraspinal extension and few cases of retroperitoneal teratomas associated with spinal dysraphism have been reported. Teratomas consist of tissues arising from all three embryonic layers. However, mature renal tissues in the form of glomeruli and tubules are sparingly found in teratomas. A 15-day-old female presented with spina bifida occulta and on evaluation a cystic presacral mass was detected. Intraoperatively the cyst was found densely adherent to the hemivertebrae but not entering the spinal canal. Histopathological examination confirmed a mature cystic teratoma but also demonstrated presence of mature renal elements in the cyst wall. The teratomas lying in proximity to spine and associated with spinal dysraphism are likely to contain mature renal tissues or even nephroblastic elements. It supports the dysembryogenic model of origin of intradural teratomas from native progenitor cells rather than aberrantly migrated germ cells.

摘要

腹膜后是小儿畸胎瘤相对少见的部位。此类肿瘤很少会有椎管内延伸,仅有少数腹膜后畸胎瘤合并脊柱裂的病例报道。畸胎瘤由来源于所有三个胚胎层的组织构成。然而,在畸胎瘤中很少能发现呈肾小球和肾小管形式的成熟肾组织。一名15天大的女婴患有隐性脊柱裂,评估时发现骶前有一囊性肿物。术中发现囊肿与半椎体紧密粘连,但未进入椎管。组织病理学检查证实为成熟囊性畸胎瘤,同时还显示囊肿壁中存在成熟肾成分。位于脊柱附近且合并脊柱裂的畸胎瘤很可能含有成熟肾组织甚至肾母细胞成分。这支持了硬脊膜内畸胎瘤起源于原始祖细胞而非异常迁移的生殖细胞的胚胎发育异常模型。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/76eb/4336057/9a72f1019ebb/10-1055-s-0033-1351393-i130078cr-1.jpg

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