Warmuth-Metz Monika, Bison Brigitte, Dannemann-Stern Elke, Kortmann Rolf, Rutkowski Stefan, Pietsch Torsten
Reference Centre for Neuroradiology of the German Society for Pediatric Hematology and Oncology (GPOH), Department of Neuroradiology, University of Würzburg, Würzburg, Germany.
Neuroradiology. 2008 May;50(5):447-52. doi: 10.1007/s00234-008-0369-7.
Atypical teratoid/rhabdoid tumors (ATRT) are rare aggressive neoplasms of the CNS affecting predominantly very young children.
We retrospectively reviewed the imaging findings of 9 CT and 32 MR examinations of the brain and spine of 33 children.
Of the 33 tumors, 11 were located in the infratentorial compartment, 16 in the supratentorial compartment, 5 in both cranial compartments, and 1 in the lower thoracic spinal cord. The mean age of the children with infratentorial or infra- and supratentorial tumors was significantly lower than the mean age of the children with purely supratentorial tumors. Heterogeneity on imaging, large size and high tumor stages are striking features reflecting the aggressive nature of this histopathological entity. Although not present in the majority of children, a distinct and unusual pattern of a wavy band-like enhancement surrounding a central hypointensity was present in 12 of 32 children (38%) in whom contrast medium was used.
To the best of our knowledge this is the largest number of imaging examinations of ATRTs so far reported. A rather unusual pattern of contrast enhancement may be typical of ATRTs.
非典型畸胎样/横纹肌样肿瘤（ATRT）是中枢神经系统罕见的侵袭性肿瘤，主要影响幼儿。
我们回顾性分析了33例儿童脑部和脊柱的9次CT及32次MR检查的影像结果。
33例肿瘤中，11例位于幕下腔，16例位于幕上腔，5例同时位于颅腔两个部位，1例位于下胸段脊髓。幕下或幕下及幕上肿瘤患儿的平均年龄显著低于单纯幕上肿瘤患儿的平均年龄。影像上的异质性、大尺寸和高肿瘤分期是反映这种组织病理学实体侵袭性的显著特征。虽然大多数儿童不存在这种情况，但在使用造影剂的32例儿童中有12例（38%）出现了一种独特且不寻常的模式，即围绕中心低信号有波浪状带状强化。
据我们所知，这是迄今为止报道的ATRT影像检查数量最多的。一种相当不寻常的造影剂强化模式可能是ATRT的典型表现。
