Tez Selda, Köktener Asli, Güler Gülnur, Ozişik Pinar
Fatih University, Radiology Department, Ankara, Turkey.
Turk Neurosurg. 2008 Jan;18(1):30-4.
Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant embryonal central nervous system (CNS) tumor, manifesting in children, and composed of rhabdoid cells, with or without fields resembling a classical primitive neuroectodermal tumor (PNET), epithelial tissue and neoplastic mesenchyme. Around 200 cases of CNS AT/RT have been documented in the literature. Although the clinical and pathological findings have been defined in large series previously, and AT/RT has become increasingly recognized, awareness of typical AT/RT is important in making the correct diagnosis of this uncommon but probably underdiagnosed entity. Neuroradiologists rarely mention AT/RT in their differential diagnosis and this paper presents two additional cases in which clinical and pathological findings are combined with neuroradiological presentation.
非典型畸胎样/横纹肌样瘤(AT/RT)是一种恶性胚胎性中枢神经系统(CNS)肿瘤,多见于儿童,由横纹肌样细胞组成,可有或无类似经典原始神经外胚层肿瘤(PNET)、上皮组织和肿瘤性间充质的区域。文献中已记录了约200例CNS AT/RT病例。尽管先前已有大量关于其临床和病理表现的报道,且AT/RT也越来越受到认可,但认识典型的AT/RT对于正确诊断这种罕见但可能诊断不足的疾病很重要。神经放射科医生在鉴别诊断中很少提及AT/RT,本文介绍另外两例将临床、病理表现与神经放射学表现相结合的病例。