Aims To describe the clinical features, imaging characteristics, histopathology, treatment and outcomes of intraocular medulloepithelioma. Methods Medical records of 11 patients with clinically or histopathologically confirmed medulloepithelioma were retrieved and reviewed. Clinical features, diagnostic challenges, imaging characteristics, management, histopathology and prognosis were assessed. Results The median age of the patients at initial diagnosis was 4 years, with the most common manifestations being leukocoria (five eyes), loss of vision (four eyes), ocular pain (one eye) and ophthalmic screening (one eye). The clinical signs include a grey-white ciliary body lesion, cataract or lens subluxation, secondary glaucoma and evident cysts. The ultrasound biomicroscopy (UBM) imaging most commonly displays ciliary body mass with intratumoural cysts (nine eyes). Three patients underwent surgery for cataract or glaucoma while the tumours were incidentally found. Two of the three patients managed by eye preserve treatments eventually required enucleation because of local tumour recurrence or phthisis. One patient treated with intra-arterial chemotherapy and cryotherapy had successful tumour regression and globe salvage. Conclusions Initial misdiagnosis, delay in diagnosis and subsequent misdirected management is not uncommon in medulloepithelioma. The presence of multiple cysts in the tumour and retrolental neoplastic cyclitic membrane detected by UBM can offer certain information. Selective intra-arterial melphalan may prevent further tumour growth, but longer follow-up is necessary until treatment efficacy is fully evaluated. Data sharing not applicable as no datasets generated and/or analysed for this study.

中文翻译：

---

眼内髓质上皮瘤11例临床特点及治疗

目的 描述眼内髓质上皮瘤的临床特征、影像学特征、组织病理学、治疗和结果。方法检索并回顾11例经临床或组织病理学证实的髓上皮瘤患者的病历。评估了临床特征、诊断挑战、影像学特征、治疗、组织病理学和预后。结果 患者初诊时中位年龄为4岁，最常见的表现为白瞳（5只眼）、视力丧失（4只眼）、眼痛（1只眼）和眼科筛查（1只眼）。临床症状包括灰白色睫状体病变、白内障或晶状体半脱位、继发性青光眼和明显的囊肿。超声生物显微镜 (UBM) 成像最常显示睫状体肿块和瘤内囊肿（九只眼睛）。3名患者因白内障或青光眼接受手术，但偶然发现了肿瘤。经过眼睛保护治疗的三名患者中，有两名最终因局部肿瘤复发或肺痨而需要摘除眼球。一名接受动脉内化疗和冷冻治疗的患者成功实现了肿瘤消退和全球挽救。结论 髓上皮瘤中最初的误诊、延误诊断以及随后的错误治疗并不少见。UBM 检测到肿瘤中存在多个囊肿和晶状体后肿瘤性环状膜可以提供一定的信息。选择性动脉内注射马法兰可能会阻止肿瘤进一步生长，但需要更长时间的随访，直到充分评估治疗效果。数据共享不适用，因为本研究没有生成和/或分析数据集。