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Intraocular medulloepithelioma clinical features and management of 11 cases
  1. Xiuqian Yi1,
  2. Fengxi Meng1,
  3. Yingwen Bi2,
  4. Lin He3,
  5. Jiang Qian1,
  6. Kang Xue1
  1. 1 Department of Ophthalmology, Fudan University Eye Ear Nose and Throat Hospital, Shanghai, China
  2. 2 Department of Pathology, Fudan University Eye Ear Nose and Throat Hospital, Shanghai, China
  3. 3 Department of Pathology, The University of Texas Southwestern Medical Center at Dallas Library, Dallas, Texas, USA
  1. Correspondence to Dr Kang Xue, Department of Ophthalmology, Fudan University Eye Ear Nose and Throat Hospital, Shanghai 200031, China; xuekang{at}foxmail.com; Professor Jiang Qian; qianjiang58{at}vip.126.com

Abstract

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.

  • ciliary body
  • neoplasia
  • treatment surgery
  • pathology

Data availability statement

Data sharing not applicable as no datasets generated and/or analysed for this study.

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Data availability statement

Data sharing not applicable as no datasets generated and/or analysed for this study.

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Footnotes

  • XY and FM contributed equally.

  • Contributors Study concept and design: KX, XY and JQ. Acquisition, analysis or interpretation of data: KX, XY and FM; Statistical analysis: XY and FM; Funding acquisition and supervision: KX; Administrative, technical or material support: YB, LH and JQ. KX and JQ are guarantors.

  • Funding Project supported by the Shanghai Committee of Science and Technology, China (Grant No.20Y11911200).

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.