儿童眼眶腺泡状横纹肌肉瘤1例并文献复习  

Pediatric orbital alveolar rhabdomyosarcoma:A case report and literature review

作　　者：张曼西 李晨阳 田甜 郭甜 黄志凤 严锐庆 刘秋芳 ZHANG Manxi;LI Chenyang;TIAN Tian;GUO Tian;HUANG Zhifeng;YAN Ruiqing;LIU Qiufang(First Department of Radiotherapy,Shaanxi Provincial Cancer Hospital,Xi’an 710000;Department of Oncology,Qian County People’s Hospital,Xianyang Shaanxi 713300,China)

机构地区：[1]陕西省肿瘤医院放疗一病区,西安710000 [2]乾县人民医院肿瘤科,陕西咸阳713300

出　　处：《临床与病理杂志》2023年第12期2206-2212,共7页Journal of Clinical and Pathological Research

摘　　要：横纹肌肉瘤(rhabdomyosarcoma,RMS)是一种罕见的恶性肿瘤,起源于骨骼肌,在儿童和青年人中多见。本研究回顾性分析1例于2019年4月23日陕西省肿瘤医院收治的眼眶部腺泡状横纹肌肉瘤(alveolar rhabdomyosarcoma,ARMS)患儿的临床资料,并对其治疗方式及结果进行分析。患儿因副鼻窦右侧眼眶内不规则软组织肿块入院。磁共振成像(magnetic resonance imaging,MRI)检查示肿块大小约为3.6 cm×5.9 cm×4.5 cm,增强扫描呈不均匀强化。免疫组织化学结果示:波形蛋白、结蛋白、成肌分化因子1、肌细胞生成素、周围神经雪旺氏细胞特异性标记S-100均为阳性,细胞核内分裂增殖相关蛋白Ki-67为40%阳性,细胞角蛋白、平滑肌肌动蛋白、胶质纤维酸性蛋白、神经元特异核蛋白、神经元特异性烯醇化酶、嗜铬素A、突触素、淋巴网状细胞一线标志抗体白细胞共同抗原、单链跨膜蛋白CD34、胎盘碱性磷酸酶、细胞表面糖蛋白CD99均为阴性。病理报告为右侧眼眶小圆细胞肿瘤,结合免疫组织化学的结果,符合ARMS。患儿于2019年4月26日予以“长春新碱+环磷酰胺+表阿霉素(vincristine+cyclophosphamide+epirubicin,VCE)”方案化学治疗8个周期,并于第2个周期予以同步放射治疗,后予以安罗替尼靶向治疗2个周期,治疗结束后疗效评价为完全缓解,治疗后至今无复发征象。Rhabdomyosarcoma(RMS)is a rare malignancy that originates from skeletal muscle,predominantly occurring in children and young adults.This study retrospectively analyzes the clinical data of a pediatric patient with orbital alveolar rhabdomyosarcoma(ARMS),admitted to Shaanxi Provincial Cancer Hospital on April 23,2019.The treatment methods and outcomes are also examined.The patient presented with an irregular soft tissue mass in the right orbital cavity,involving the paranasal sinus.Magnetic resonance imaging(MRI)showed a mass size of approximately 3.6 cm×5.9 cm×4.5 cm,with heterogeneous enhancement on contrast imaging.Immunohistochemical results showed positive staining for vimentin,desmin,myogenic differentiation 1,myogenin,and peripheral nerve Schwann cell-specific marker S-100,with intracellular fission and proliferation-related protein Ki-67 of 40%positive.Negative staining was observed for cytokeratin,smooth muscle actin,glial fibrillary acidic protein,neuron-specific nuclear protein,neuron-specific enolase,chromogranin A,synaptophysin,leucocyte common antigen,single-chain transmembrane protein CD34,placental alkaline phosphatase,and cell surface glycoprotein CD99.Pathological reports described a small round cell tumor in the right orbital region,consistent with acinar-type RMS(ARMS)based on immunohistochemistry.The patient received eight cycles of chemotherapy with the“vincristine+cyclophosphamide+epirubicin(VCE)”regimen starting on April 26,2019,and synchronous radiotherapy during the second cycle.Subsequently,two cycles of anlotinib-targeted therapy were administered.The treatment was concluded with a complete response,and no signs of recurrence have been observed to date.

关 键 词：儿童 眼眶 腺泡状横纹肌肉瘤 

分 类 号：R739.72[医药卫生—肿瘤]