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一名5岁儿童的弥漫性葡萄膜黑色素瘤。

Diffuse uveal melanoma in a 5-year-old child.

作者信息

Rosenbaum P S, Boniuk M, Font R L

机构信息

Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine, Houston, Texas 77030.

出版信息

Am J Ophthalmol. 1988 Nov 15;106(5):601-6. doi: 10.1016/0002-9394(88)90594-6.

DOI:10.1016/0002-9394(88)90594-6
PMID:3189476
Abstract

A 5-year-old white boy developed a gray-tan discoloration with nodular thickening of the iris stroma in his left eye six weeks earlier. Ophthalmologic examination demonstrated keratic precipitates, peripheral anterior synechiae, and several pale-tan choroidal infiltrates in the involved eye. Cytologic examination of the aqueous humor and results of iris biopsy were interpreted as malignant melanoma of the iris, predominantly of the epithelioid cell type. The globe was enucleated one week later. Histopathologic examination disclosed a diffuse malignant melanoma of the uvea, mixed cell type, with multiple cysts of the ciliary epithelium. Study by electron microscopy indicated bizarre neoplastic melanocytes containing numerous immature melanosomes in various stages of melanogenesis, as well as fully melanized mature melanosomes. Eight months after enucleation the patient developed metastasis to the nasopharynx and base of the skull.

摘要

一名5岁白人男孩六周前左眼虹膜基质出现灰棕色变色并伴有结节状增厚。眼科检查发现患眼角膜后沉着物、周边前粘连以及几个淡棕色脉络膜浸润灶。房水的细胞学检查及虹膜活检结果诊断为虹膜恶性黑色素瘤,主要为上皮样细胞型。一周后眼球被摘除。组织病理学检查显示为葡萄膜弥漫性恶性黑色素瘤,混合细胞型,伴有睫状体上皮多处囊肿形成。电子显微镜检查发现异常的肿瘤性黑素细胞,含有处于黑素生成不同阶段的大量未成熟黑素小体以及完全黑素化的成熟黑素小体。眼球摘除八个月后,患者出现鼻咽部及颅底转移。

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