3例累及邻近多部位的儿童鼻腔鼻窦胚胎型横纹肌肉瘤临床分析
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吴振恭,Email:wzg6688@163.com


Clinical analysis of embryonic rhabdomyosarcoma invading the surrounding multiple structures in nasal cavity and sinuses of children: report of 3 cases
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    目的探讨儿童横纹肌肉瘤(rhabdomyosarcoma,RMS)的临床特点、影像学检查及临床病理学特征,以期减少漏诊误诊,提高临床诊断和鉴别诊断水平。方法回顾性分析3例不典型学龄前期儿童RMS的详细临床资料并复习相关文献。结果2例以眼部症状为主诉收入院,1例罕见以反复鼻腔出血并睡觉打鼾为主诉收入院,相关影像学检查显示肿瘤多已广泛侵犯破坏周围结构,行相关手术病理确诊,均为胚胎性RMS。免疫组化显示3例患者Myogenin均为阳性,Ki67(LIt分别约为90%、80%、70%),其中2例VIM阳性,2例DES(部分或者局灶性)阳性,2例CD99阳性。后转入肿瘤科规律治疗。结论儿童RMS早期临床表现不典型呈现多样化,具有恶性程度高、侵袭性强、进展快等特点,需要根据临床特征结合影像学检查及免疫组化结果确诊。

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    Abstract:ObjectiveTo investigate the clinical, imaging examination and clinicopathological features of rhabdomyosarcoma in children, so as to reduce the misdiagnosis and missed diagnosis for improving clinical diagnosis and differential diagnosis.MethodsThe clinical data of 3 cases of rhabdomyosarcoma in atypical preschool children were retrospectively analyzed and the related literatures were reviewed.Results2 cases complained mainly of ocular symptoms, 1 case complained mainly of recurrent nasal bleeding and snoring. The imaging examination showed that most of the tumors had invaded and destroyed the surrounding structures, and most of them were diagnosed for embryonic rhabdomyosarcoma by operation and pathology. Immunohistochemistry showed that Myogenin was positive in all 3 patients, Ki67 (LIt was about 90%, 80% and 70%, respectively), 2 patients were positive for VIM, 2 patients were positive for DES (partial or focal), and 2 patients were positive for CD99. Then,they were transferred to the department of oncology, and treated regularly.ConclusionsThe early clinical manifestations of rhabdomyosarcoma in children are atypical and diversified. It has the characteristics of high malignancy, strong invasiveness and rapid progress. The diagnosis needs to be confirmed according to the clinical features, combined with imaging examination and immunohistochemical results.

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吴振恭,熊俊,周良强,陈请国,陈金,褚汉启,陶雁玲,甄宏韬,王丽.3例累及邻近多部位的儿童鼻腔鼻窦胚胎型横纹肌肉瘤临床分析[J].中国耳鼻咽喉颅底外科杂志,2019,25(5):546-550

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