Phakomatous choristoma may be located in the eyelid or orbit or both
Phakomatous choristoma may be located in the eyelid or orbit or both
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DOI：
10.1034/j.1600-0420.1999.770320.x
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发表时间：
1999-06-01
期刊：
影响因子：
--
通讯作者：
Seregard, S
中科院分区：
文献类型：
--
作者：
Seregard, S
目的：晶状体瘤性脉络膜瘤是一种罕见的先天性晶状体肿瘤。在临床表现的确切位置是有争议的，但在这里，我们提供的证据表明，phakomatous绒毛膜瘤可能位于下眼睑或眼眶或both.Methods：病例报告的婴儿提出了一个肿块在下眼睑出生时，系统回顾以往的情况下，phakomatous绒毛膜瘤和轮廓的组织病理学特征，在正常的中面部胚胎发育在不同的胎龄。本病例之组织病理学特徴与一个有鳞瘤性绒毛膜瘤相符。术前影像学检查和手术时的临床表现表明，肿瘤占据了下眼睑和前眼眶的部分。正常人胚胎的组织学切片显示，在26-mm的发育阶段，胚胎透镜形成，但骨壁定义的轨道尚未present.Conclusion：Phakomatous chorioma出现在未分化的间充质组织，后来可能会发展到下眼睑或眼眶取决于choriostomatous元素沉积浅或深的胚胎表面的设置。
Purpose: Phakomatous choristoma is a rare congenital tumour of lenticular origin. The exact location at clinical presentation is controversial, but herein we provide evidence that phakomatous choristoma may be located in the lower eyelid or orbit or both.Methods: Case report of an infant presenting with a mass in the lower eyelid at birth, a systematic review of previous cases of phakomatous choristoma and an outline of the histopathological features in normal mid-facial embryonic development at different gestational ages.Results: The histopathological features of the present case were consistent with those of a phakomatous choristoma. The preoperative imaging studies and clinical findings at surgery suggested that the tumour occupied parts of both the lower eyelid and anterior orbit. Histopathological sections of a normal human embryo showed that at the 26-mm stage of development the embryonic lens is formed but the bony walls defining the orbit are not yet present.Conclusion: Phakomatous choristoma arises in a setting of undifferentiated mesenchymal tissue which later may develop into the lower eyelid or orbit depending on the choristomatous elements being deposited superficial or deep to the embryonic surface.