Atypical teratoid/rhabdoid tumor (ATRT) arising from the 3rd cranial nerve in infants: a clinical-radiological entity?

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• 作者：Christopher C. Oh ; Brent A. Orr ; Bruno Bernardi…
• 关键词：Atypical teratoid rhabdoid tumor ; Oculomotor nerve palsy ; CN III ; Magnetic resonance imaging ; Diffusion ; weighted imaging
• 刊名：Journal of Neuro-Oncology
• 出版年：2015
• 出版时间：September 2015
• 年：2015
• 卷：124
• 期：2
• 页码：175-183
• 全文大小：1,443 KB
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• 作者单位：Christopher C. Oh (1) (2)
  Brent A. Orr (3)
  Bruno Bernardi (4)
  Maria Luisa Garr茅 (5)
  Andrea Rossi (5)
  Lorenzo Fig脿-Talamanca (4)
  Giles W. Robinson (6)
  Zolt谩n Patay (1)
  
  1. Department of Radiological Sciences, St. Jude Children鈥檚 Research Hospital, Memphis, TN, USA
  2. Department of Radiology, University of Tennessee Health Sciences Center, Memphis, TN, USA
  3. Department of Pathology, St. Jude Children鈥檚 Research Hospital, Memphis, TN, USA
  4. Department of Neuroradiology, Bambino Ges霉 Children鈥檚 Hospital, Rome, Italy
  5. Neurooncology Unit, Istituto Giannina Gaslini, Genoa, Italy
  6. Department of Oncology, St. Jude Children鈥檚 Research Hospital, Memphis, TN, USA
  
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Oncology
  
• 出版者：Springer Netherlands
• ISSN：1573-7373

文摘

Atypical teratoid/rhabdoid tumor (ATRT) is a rare embryonal tumor of the central nervous system with preponderance in very young children, the majority of whom are younger than 3 years of age at diagnosis. Historically, outcomes of this aggressive disease, even with extensive multimodal therapy, have been dismal. Recent improvements have come from therapies directed exclusively towards ATRT, but misdiagnosis or delays in the correct diagnosis lead to significantly worse survival rates. ATRTs most commonly occur supratentorially but have been described in virtually all central nervous system locations, including the cerebellopontine angle cistern, meninges, and spinal canal, and extradural locations. ATRTs originating from cranial nerves are rare. Here, we describe three cases of solitary ATRT arising from the 3rd cranial nerve (CN III) or close to its origin in the midbrain, all of which presented in patients within 6 months of birth, with isolated unilateral oculomotor nerve palsy and strikingly similar magnetic resonance imaging (MRI) features. These MRI features include IV contrast enhancement, relative T2 hyposignal, and restricted water diffusion on apparent diffusion coefficient images, findings which are consistent with angiogenesis and high cellularity, and hence, suggestive of malignancy. We conclude that ATRT should be placed high on the differential diagnosis list when encountering a young infant presenting with isolated, unilateral 3rd nerve palsy and a small, solitary tumor arising from CN III that demonstrates malignant conventional and diffusion-weighted imaging features on MRI. Keywords Atypical teratoid rhabdoid tumor Oculomotor nerve palsy CN III Magnetic resonance imaging Diffusion-weighted imaging