Purpose: 

To document a case of phakomatous choristoma (PC), a rare benign periocular tumor, and to review the literature on previously reported cases.

Methods: 

The authors describe a case of PC and its clinical, histopathological, immunohistochemical, and radiological features, and present findings from a comprehensive review of all previously reported cases of this rare pediatric tumor.

Results: 

This case report and review highlights the benign clinical nature of PC. It typically presents at birth as a lower eyelid mass involving the orbit. Definitive diagnosis is made with hematoxylin and eosin stain showing the tumor’s histological similarities to lenticular tissue.

Conclusion: 

PC remains a rare entity that should be included in the differential of pediatric eyelid lesions. Surgical excision is curative, and the postoperative clinical course is unremarkable as there have been no reports of recurrence. Prompt recognition and surgical intervention may be warranted due to astigmatism and anisometropia induced by mass effect.

中文翻译：

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Phakomatous Choristomas：病例报告和文献回顾

目的： 

记录一例 phakomatous choristoma (PC)，一种罕见的良性眼周肿瘤，并回顾先前报道的病例的文献。

方法： 

作者描述了一个 PC 病例及其临床、组织病理学、免疫组织化学和放射学特征，并介绍了对所有先前报道的这种罕见儿科肿瘤病例的全面回顾的结果。

结果： 

本病例报告和回顾突出了 PC 的良性临床性质。它通常在出生时表现为涉及眼眶的下眼睑肿块。明确诊断是通过苏木精和伊红染色显示肿瘤的组织学与晶状体组织相似。

结论： 

PC 仍然是一个罕见的实体，应包括在儿童眼睑病变的鉴别中。手术切除是治愈性的，术后临床病程不显着，因为没有复发的报告。由于肿块效应引起的散光和屈光参差，可能需要及时识别和手术干预。