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一名儿科患者上颌窦胚胎性横纹肌肉瘤伴眼眶受累:病例报告

Embryonal rhabdomyosarcoma in the maxillary sinus with orbital involvement in a pediatric patient: Case report.

作者信息

de Melo Ana Carolina Rodrigues, Lyra Tácio Candeia, Ribeiro Isabella Lima Arrais, da Paz Alexandre Rolim, Bonan Paulo Rogério Ferreti, de Castro Ricardo Dias, Valença Ana Maria Gondim

机构信息

Department of Clinical and Social Dentistry, Federal University of Paraíba, João Pessoa 58051-900, Brazil.

Department of Pathology, Hospital Napoleão Laureano, João Pessoa 58015-170, Brazil.

出版信息

World J Clin Cases. 2017 Dec 16;5(12):440-445. doi: 10.12998/wjcc.v5.i12.440.

Abstract

This report presents a case of embryonal rhabdomyosarcoma (eRMS) located in the left maxillary sinus and invading the orbital cavity in a ten-year-old male patient who was treated at a referral hospital. The images provided from the computed tomography showed a heterogeneous mass with soft-tissue density, occupying part of the left half of the face inside the maxillary sinus, and infiltrating and destroying the bone structure of the maxillary sinus, left orbit, ethmoidal cells, nasal cavity, and sphenoid sinus. An analysis of the histological sections revealed an undifferentiated malignant neoplasm infiltrating the skeletal muscle tissue. The immunohistochemical analysis was positive for the antigens: MyoD1, myogenin, desmin, and Ki67 (100% positivity in neoplastic cells), allowing the identification of the tumour as an eRMS. The treatment protocol included initial chemotherapy followed by radiotherapy and finally surgery. The total time of the treatment was nine months, and in 18-mo of follow-up period did not show no local recurrences and a lack of visual impairment.

摘要

本报告介绍了一例发生于一名10岁男性患者左上颌窦并侵犯眼眶的胚胎性横纹肌肉瘤(eRMS)病例,该患者在一家转诊医院接受治疗。计算机断层扫描提供的图像显示,有一个软组织密度不均匀的肿块,占据上颌窦内左半侧面部的一部分,并浸润和破坏上颌窦、左眼眶、筛窦、鼻腔和蝶窦的骨结构。组织学切片分析显示为未分化恶性肿瘤浸润骨骼肌组织。免疫组化分析显示抗原MyoD1、肌细胞生成素、结蛋白和Ki67呈阳性(肿瘤细胞中阳性率为100%),从而确定该肿瘤为eRMS。治疗方案包括初始化疗,随后放疗,最后手术。治疗总时长为9个月,在18个月的随访期内未出现局部复发且无视力损害。

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