Imaging features of medulloepithelioma: report of four cases and review of the literature

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• 作者：Rakhee K. Sansgiri ; Matthew Wilson ; M. Beth McCarville…
• 关键词：Medulloepithelioma ; Intraocular ; Teratoid ; Ciliary body ; Children
• 刊名：Pediatric Radiology
• 出版年：2013
• 出版时间：October 2013
• 年：2013
• 卷：43
• 期：10
• 页码：1344-1356
• 全文大小：946KB
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  4. Brennan RC, Wilson MW, Kaste S et al (2012) US and MRI of pediatric ocular masses with histopathological correlation. Pediatr Radiol 42:738-49 CrossRef
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• 作者单位：Rakhee K. Sansgiri (1) (2)
  Matthew Wilson (1) (3) (4)
  M. Beth McCarville (1) (5)
  Kathleen J. Helton (1)
  
  1. Department of Radiological Sciences, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, MS 220, Memphis, TN, 38105-3678, USA
  2. Department of Diagnostic Radiology, Le Bonheur Children’s Hospital, Memphis, TN, USA
  3. Departments of Surgery and Pathology, St. Jude Children’s Research Hospital, Memphis, TN, USA
  4. Department of Ophthalmology, University of Tennessee Health Science Center, College of Medicine, Memphis, TN, USA
  5. Department of Radiology, University of Tennessee Health Science Center, College of Medicine, Memphis, TN, USA
  
• ISSN：1432-1998

文摘

Background Intraocular medulloepithelioma is a childhood tumor arising from the nonpigmented primitive ciliary neuroepithelium. Although rarer than retinoblastoma, it remains the second most common primary intraocular neoplasm in children. The rarity of intraocular medulloepithelioma creates the challenge in establishing a clinical diagnosis, and radiologically the tumor is often confused with other intraocular masses. Objective To describe the clinical, imaging and pathological features of intraocular medulloepithelioma with emphasis on the role of imaging to enable its differentiation from more common intraocular pathology. Materials and methods We retrospectively analyzed the clinical, histopathological and imaging data of four children with intraocular medulloepithelioma. Results All four children had medulloepithelioma arising from the ciliary body. The children were imaged with US (n--), MRI (n--), whole-body 99mTc-MDP scintigraphy (n--) and CT (n--). All four children had enucleation of the involved eye. One tumor was a malignant teratoid variant, two tumors were malignant nonteratoid variants and one was a nonteratoid variant of uncertain malignant potential. None of the tumors had extraocular extension on histopathology or imaging. Two children had associated retinal detachment on US and MRI examinations. All tumors were iso/hyperintense to vitreous on T1-weighted and hypointense on T2-weighted MRI and showed marked contrast enhancement of the solid components. No calcifications were identified on US or CT examinations. Conclusion Our findings are consistent with previously reported cases of medulloepithelioma. This series emphasizes the roles of various imaging modalities, with pathological correlation, in differentiating the tumor from other ciliary body masses, in detecting tumor extension and in identifying associated ocular complications. In this series we also describe the results of postsurgical follow-up for tumor recurrence.