并发眼阵挛肌阵挛综合征的神经母细胞瘤影像学特征及其在早期诊断中的价值  

Imaging features of neuroblastoma complicated with opsoclonus myoclonus syndrome and its early diagnostic value

作　　者：寿然 王霄英[1] 孙青[2] 谢瑶[2] 吴鹏辉[2] 赵卫红[2] SHOU Ran;WANG Xiaoying;SUN Qing;XIE Yao;WU Penghui;ZHAO Weihong(Department of Radiology,First Hospital,Peking University,Beijing 100034,China;Department of Pediatric,First Hospital,Peking University,Beijing 100034,China)

机构地区：[1]北京大学第一医院影像科,北京100034 [2]北京大学第一医院儿科,北京100034

出　　处：《中国小儿血液与肿瘤杂志》2023年第3期153-158,共6页Journal of China Pediatric Blood and Cancer

摘　　要：目的探讨并总结儿童神经母细胞瘤并发眼阵挛肌阵挛综合征(NB-OMS)的肿物影像学特征,以指导临床早期诊断,避免误诊。方法采用病例对照研究,以北京大学第一医院2007年1月—2022年12月诊断的NB-OMS患儿为病例组,按照1∶1的比例,收集同时期、同一家医院诊治、同年龄(月龄相差<6个月)、同性别的单纯神经母细胞瘤(NB)患儿为对照组,影像学资料由两位影像科医师重新读片分析。结果(1)NB-OMS组患儿34例,男21例,女13例,发病中位年龄为18(6-48)个月;对照组发病中位年龄为20(8-50)个月。(2)所有NB-OMS患儿都因增强CT和/或MRI检查发现肿物而得到临床诊断,手术切除肿物病理证实诊断。(3)NB-OMS组患儿肿物位于肾上腺者仅占17.6%,其余均位于脊柱旁,其中位于胸椎旁、腰椎旁和骶椎旁者分别占32.4%、38.2%和11.8%;单纯NB组肿物位于肾上腺者占55.9%,其余分别位于胸椎旁(14.7%)、腰椎旁(14.7%)、骶椎旁(2.9%)和颈椎旁(11.8%)(P<0.01)。(4)与单纯NB组肿物影像特征相比,NB-OMS组肿物最大直径≥5cm者较少,边界清楚和实性者较多,钙化较少,包绕周围重要血管者较少,无远处转移。(5)NB-OMS组尿香草杏仁酸阳性率仅为11.8%,尿高香草酸阳性率为2.9%,血清特异性烯醇化酶≥100ng/mL者仅占2.9%,明显低于单纯NB组(分别为为76.5%、82.6%和64.8%)(P<0.01)。(6)长期随访,NB-OMS组有1例死亡,肿物位于肾上腺;单纯NB组有11例死亡,其中8例原发肿瘤位于肾上腺,死亡原因均为肿瘤进展或复发。结论NB-OMS患儿肿物多发生在脊柱旁交感神经链,而不是肾上腺,肿瘤标记物在NB-OMS中阳性率非常低,胸腹盆增强CT/MRI检查对NB-OMS的早期诊断具有重要意义。肿瘤发生在肾上腺者可能预后不佳。Objective To investigate and summarize the imaging features of pediatric neuroblastoma complicated with ocular myoclonus syndrome(NB-OMS),to help early clinical diagnosis.Methods A case-control study was conducted.Patients with NB-OMS diagnosed in Peking University First Hospital from January 2007 to December 2022 were selected as the case group.Patients with the same age(less than 6 months)and of the same gender with neuroblastoma(NB)at the same time and in the same hospital were selected as the control group.The imaging data were reviewed separately by two radiologists.Results①There were thirty-four patients in NB-OMS group,including 21 males and 13 females,with a median age of onset of 18(6-48)months.The number of patients in the NB group and the ratio of males to females were the same as those in the NB-OMS group,and the median age of onset was 20(8-50)months.②All NB-OMS patients were diagnosed by a masse found by contrast-enhanced CT and/or MRI,followed by surgical resection of the tumor and pathological confirmation of the diagnosis.③The results of imaging examination showed that only 17.6% of the tumors in the NB-OMS group were located in the adrenal gland,and the rest(83.4%)were located in the paraspinal area,including 32.4%in the thoracic paraspinal area,38.2% in the lumbar paraspinal area,and 11.8% in the sacral paraspinal area;In NB group,55.9% of patients had tumors located in adrenal gland,and the rest were located in thoracic paravertebral area(14.7%),lumbar paravertebral area(14.7%),sacral paravertebral area(2.9%)and cervical paravertebral area(11.8%)(P<0.01).④In comparison with NB group,the masses of NB-OMS cases were mostly smaller in size,more clearly boundary and solid,less calcified and,and less defined in L2,none in M of INRG staging system.⑤The positive rate of urinary VMA in NB-OMS group was only 11.8%,the VHA was 2.9%,and the serum NSE≥100ng/mL was only 2.9%.It was significantly lower than that in the NB group(76.5%,82.6% and 64.8%,respectively)(P<0.01).⑥In the long-term foll

关 键 词：眼阵挛肌阵挛综合征 神经母细胞瘤 儿童 影像学 早期诊断 

分 类 号：R739.4[医药卫生—肿瘤]