Department of Diagnostic Radiology, Section of Neuroradiology, University of Texas MD Anderson Cancer Center, Houston, TX.
Department of Pathology, Section of Neuropathology, University of Texas MD Anderson Cancer Center, Houston, TX.
J Neuroimaging. 2018 Sep;28(5):483-489. doi: 10.1111/jon.12524. Epub 2018 May 24.
Embryonal tumor with multilayered rosettes (ETMR), C19MC-altered, is a recently described, rare central nervous system tumor. To our knowledge, the imaging findings of this tumor have not been systematically evaluated in the neuroradiology literature. We present here the clinical, radiological, and pathological correlation of a case series of this very rare tumor, including the full range of anatomic compartment presentations (supratentorial, infratentorial, and spinal).
We retrospectively analyzed 7 (4M, 3F) pathologically-proven cases of ETMR referred to our institution between 2007 and 2017. We demonstrate the imaging characteristics of this tumor on CT and MRI with advanced imaging.
All of the patients are children (ages 1-12). On MR imaging of ETMR, contrast enhancement is often heterogeneous and minimal if any, and there is no significant surrounding T2 fluid-attenuated inversion recovery (FLAIR) hyperintensity to suggest edema. The lesions were often expansile with no evidence of infiltration of the fiber tracks that were displaced by the tumor mass. Diffusion-weighted imaging often demonstrated restricted diffusion within ETMRs. On magnetic resonance spectroscopy (MRS), the choline/creatine (Cho/Cr) ratio is increased, with low N-acetylaspartate (NAA) or NAA/Cho ratio, typical of high-grade tumors.
We demonstrate the conventional and advanced imaging characteristics of ETMR, including MRS and diffusion tensor imaging, which, to our knowledge, have not been systematically evaluated in the radiology literature. The knowledge gained may potentially impact patient management, especially in inoperable cases and in locations where it is risky to perform a biopsy.
胚胎性肿瘤伴多层菊形团（ETMR），C19MC 改变，是一种最近描述的罕见中枢神经系统肿瘤。据我们所知，这种肿瘤的影像学表现尚未在神经放射学文献中得到系统评估。我们在此介绍了该非常罕见肿瘤的一系列病例的临床、放射学和病理学相关性，包括其所有解剖部位表现（幕上、幕下和脊髓）。
我们回顾性分析了 2007 年至 2017 年间我院确诊的 7 例（4 例男性，3 例女性）经病理证实的 ETMR 病例。我们展示了该肿瘤在 CT 和 MRI 上的影像学特征，包括先进的影像学表现。
所有患者均为儿童（年龄 1-12 岁）。在 ETMR 的磁共振成像（MRI）上，增强后常呈不均匀强化，且强化程度轻微，如果有强化，周围 T2 液体衰减反转恢复（FLAIR）高信号也无明显水肿。病变通常呈膨胀性生长，无纤维束浸润的迹象，肿瘤压迫纤维束使之移位。弥散加权成像（DWI）常显示 ETMR 内弥散受限。磁共振波谱（MRS）上，胆碱/肌酸（Cho/Cr）比值升高，N-乙酰天门冬氨酸（NAA）或 NAA/Cho 比值降低，这是高级别肿瘤的典型特征。
我们展示了 ETMR 的常规和先进的影像学特征，包括 MRS 和弥散张量成像，据我们所知，这些特征尚未在放射学文献中得到系统评估。这些知识可能会对患者的管理产生影响，特别是在无法手术的病例和进行活检风险较高的部位。
