Department of Neurology, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, One children's Way, Little Rock, AR 72202, USA.
J Neurooncol. 2010 Jul;98(3):431-4. doi: 10.1007/s11060-009-0094-z. Epub 2009 Dec 19.
Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics. AT/RT typically involves the posterior fossa of the pediatric population. Supratentorial AT/RT is exceedingly rare. In this report, we describe a very unusual case of a child who presented with signs and symptoms suggestive of leptomeningitis. However, imaging studies and histologic findings showed plaque-like AT/RT involving the leptomeninges of the cerebrum, cerebellum, and spinal cord. The disease proved to be rapidly fatal and resulted in the patient's death within approximately two weeks. To our knowledge, this is the first case of primary leptomeningeal AT/RT involving the supratentorial leptomeninges.
中枢神经系统的非典型畸胎样横纹肌样肿瘤（AT/RT）最近被描述为一种具有特征性形态、免疫表型和分子特征的独特临床病理实体。AT/RT 通常涉及小儿的后颅窝。幕上 AT/RT 极为罕见。在此报告中，我们描述了一例非常不寻常的患儿，其表现出脑膜脑炎的体征和症状。然而，影像学研究和组织学发现显示累及大脑、小脑和脊髓软脑膜的斑块状 AT/RT。该疾病迅速致命，导致患者在大约两周内死亡。据我们所知，这是首例累及幕上软脑膜的原发性软脑膜 AT/RT。
