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眶顶孤立性骨内婴儿肌纤维瘤

Solitary intraosseous infantile myofibroma of the orbital roof.

作者信息

Shields C L, Husson M, Shields J A, Mercado G, Eagle R C

机构信息

Oncology Service, Wills Eye Hospital, Thomas Jeferson University, Philadelphia, Pa., USA.

出版信息

Arch Ophthalmol. 1998 Nov;116(11):1528-30. doi: 10.1001/archopht.116.11.1528.

Abstract

BACKGROUND

There are several rare tumors that can cause proptosis in an infant, including infantile myofibroma.

METHODS

A 3-month-old infant developed a painless, bone-destructive superomedial orbital mass, raising concern for orbital malignant neoplasms. Computed tomography disclosed a bone-destructive mass of the sphenoid wing. On magnetic resonance imaging, the intraosseous mass was well-circumscribed, surrounded by cortical bone, and showed prominent enhancement.

RESULTS

Superomedial orbitotomy and biopsy revealed a lesion composed of spindled to stellate cells, without mitotic activity, set in a fibromyxoid stroma. Immunohistochemical stains were positive for vimentin and actin. Ultrastructurally, there were actinlike thin filaments, mitochondria, and rough endoplasmic reticulum confirming a myofibroblastic proliferation and supporting the diagnosis of congenital infantile myofibroma.

CONCLUSION

Infantile myofibroma is a benign tumor that occurs rarely in the ocular region but can cause prominent bone destruction, misleading the clinician to suspect a malignant neoplasm.

摘要

背景

有几种罕见肿瘤可导致婴儿眼球突出,包括婴儿肌纤维瘤。

方法

一名3个月大的婴儿出现了一个无痛性、破坏骨质的眶内上象限肿块,引发了对眼眶恶性肿瘤的担忧。计算机断层扫描显示蝶骨翼有一个破坏骨质的肿块。在磁共振成像上,骨内肿块边界清晰,被皮质骨包围,并显示出明显强化。

结果

眶内上象限开眶术及活检显示病变由梭形细胞至星状细胞组成,无有丝分裂活性,位于纤维黏液样基质中。免疫组织化学染色波形蛋白和肌动蛋白呈阳性。超微结构显示有肌动蛋白样细丝、线粒体和粗面内质网,证实为肌成纤维细胞增殖,支持先天性婴儿肌纤维瘤的诊断。

结论

婴儿肌纤维瘤是一种良性肿瘤,很少发生于眼部区域,但可导致明显的骨质破坏,使临床医生误疑为恶性肿瘤。

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