临床与病理杂志2023，Vol.43Issue(12)：2206-2212,7.DOI:10.11817/j.issn.2095-6959.2023.230243
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
张曼西 1李晨阳 1田甜 1郭甜 1黄志凤 1严锐庆 2刘秋芳1
作者信息
- 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
- 折叠
摘要
Abstract
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Key words
children/orbit/alveolar rhabdomyosarcoma引用本文复制引用
张曼西,李晨阳,田甜,郭甜,黄志凤,严锐庆,刘秋芳..儿童眼眶腺泡状横纹肌肉瘤1例并文献复习[J].临床与病理杂志,2023,43(12):2206-2212,7.
