Journal of Otolaryngology and Ophthalmology of Shandong University ›› 2022, Vol. 36 ›› Issue (1): 55-59.doi: 10.6040/j.issn.1673-3770.0.2021.409

Previous Articles     Next Articles

A case of an infant with embryonal rhabdomyosarcoma in the middle ear and literature review

LI Ling1,2, LI Cong1,2, SUN Yan2, CHEN Liang2   

  1. 1. The second Medical College of BinZhou Medical University, Yantai 264003, Shandong, China;
    2. Department of Otorhinolaryngology & Head and Neck Surgery, Yantai Yuhuangding Hospital Affiliated to Qingdao University/ Shandong Provincial Clinical Research Center for Otorhinolaryngologic Diseases, Yantai 264000, Shandong, China
  • Published:2022-02-22

PDF (PC)

224

Abstract: Objective Raising awareness of otolaryngologists on embryonal rhabdomyosarcoma disease. Methods The clinical data of an infant with embryonal rhabdomyosarcoma in the middle ear were analyzed, and related literatures were reviewed. Results The patient was a 10-month-old female with a bleeding mass in the left external auditory canal. Computed tomography and magnetic resonance imaging revealed soft tissue density shadows in the left external auditory meatus, tympanum, and mastoid sinus that were accompanied with an enlarged lymph node in the parapharyngeal, neck and bilateral axilla. Moreover, the diagnosis of RMS was confirmed by postoperative pathological and immunohistochemical examination after biopsy. The patient died of brain metastasis four months after the end of radiation and chemotherapy. Conclusion RMS in the middle ear is rare. Due to its obscure location and lack of typical clinical manifestations in the early stage, RMS is difficult to detect and prone to misdiagnosis. The malignant degree of this disease is high. Thus, the early detection, diagnosis, and treatment of this disease should be further studied to improve the survival rate of patients.

Key words: Middle ear, Embryonic, Rhabdomyosarcoma, Infant, Malignancy

CLC Number: 

  • R764.2
[1] 中国抗癌协会小儿肿瘤专业委员会, 中华医学会儿科学分会血液学组, 中华医学会小儿外科学分会肿瘤组. 中国儿童及青少年横纹肌肉瘤诊疗建议(CCCG-RMS-2016)[J]. 中华儿科杂志, 2017, 55(10): 724-728. doi:10.3760/cma.j.issn.0578-1310.2017.10.002.
[2] Hayes SM, Biggs TC, Bird JH, et al. Rhabdomyosarcoma of the middle ear cavity presenting with Horner's syndrome[J]. Annals, 2019, 101(1): e8-e10. doi:10.1308/rcsann.2018.0157.
[3] 何健, 钱立庭, 高劲. 外耳道腺样囊性癌侵犯中耳、颞叶且伴颈部淋巴结转移一例报告[J]. 山东大学耳鼻喉眼学报, 2018, 32(6): 109-111,118. doi: 10.6040/j.issn.1673-3770.0.2017.424. HE Jian, QIAN Liting, GAO Jin. A case of external auditory canal adenoid cystic carcinoma invading the middle ear and temporal lobe, with cervical lymph node metastasis[J]. J Otolaryngol Ophthalmol Shandong Univ, 2018,32(6): 109-111,118. doi: 10.6040/j.issn.1673-3770.0.2017.424.
[4] 赵笑冰, 张大为, 陈仁杰. 中耳间变性大 T 细胞淋巴瘤一例并文献复习[J/OL].山东大学耳鼻喉眼学报,2022[2022-02-18]. doi:10.6040/j.issn.1673-3770.0.2021.318. ZHAO Xiaobing, ZHANG Dawei, CHEN Renjie. A case of middle-ear anaplastic large T- cell lymphoma and literature review[J]. J Otolaryngol Ophthalmol Shandong Univ, 2022[2022-02-18]. doi:10.6040/j.issn.1673-3770.0.2021.318.
[5] Jo VY, Fletcher CDM. WHO classification of soft tissue tumours: an update based on the 2013(4th)edition[J]. Pathology, 2014, 46(2): 95-104. doi:10.1097/PAT.0000000000000050.
[6] Leiner J, Le Loarer F. The current landscape of rhabdomyosarcomas: an update[J]. Virchows Arch, 2020, 476(1): 97-108. doi:10.1007/s00428-019-02676-9.
[7] Fischer TD, Gaitonde SG, Bandera BC, et al. Pediatric-protocol of multimodal therapy is associated with improved survival in AYAs and adults with rhabdomyosarcoma[J]. Surgery, 2018, 163(2): 324-329. doi:10.1016/j.surg.2017.10.027.
[8] Janz TA, Camilon PR, Cheung AY, et al. A review of pediatric middle ear tumors and analysis of the demographics, management, and survival of pediatric rhabdomyosarcomas of the middle ear[J]. Int J Pediatr Otorhinolaryngol, 2018, 112: 109-112. doi:10.1016/j.ijporl.2018.06.017.
[9] 李克鹏, 刘柱, 金书丞, 等. 鼻部横纹肌肉瘤诊疗分析[J]. 临床耳鼻咽喉头颈外科杂志, 2017,31(18): 1398-1401,1407. doi:10.13201/j.issn.1001-1781.2017.18.005. LI Kepeng, LIU Zhu, JIN Shucheng, et al. Clinical analysis of nose rhabdomyosarcoma[J]. J Clin Otorhinolaryngol Head Neck Surg, 2017,31(18): 1398-1401,1407. doi:10.13201/j.issn.1001-1781.2017.18.005.
[10] Hicks J, Flaitz C. Rhabdomyosarcoma of the head and neck in children[J]. Oral Oncol, 2002, 38(5): 450-459. doi:10.1016/s1368-8375(01)00105-1.
[11] 彭晓敏, 黎阳, 熊稀霖, 等. 放疗及二次根治手术对儿童横纹肌肉瘤的疗效影响分析[J]. 中国小儿血液与肿瘤杂志, 2021,26(1): 29-34. doi: 10.3969/j.issn.1673-5323.2021.01.005. PENG Xiaomin, LI Yang, XIONG Xilin, et al. Effect of radiotherapy and second radicalsurgery on rhabdomyosarcoma in children[J]. J China Pediatr Blood Cancer, 2021,26(1): 29-34. doi: 10.3969/j.issn.1673-5323.2021.01.005.
[12] Dasgupta R, Fuchs J, Rodeberg D. Rhabdomyosarcoma[J]. Semin Pediatr Surg, 2016, 25(5): 276-283. doi:10.1053/j.sempedsurg.2016.09.011.
[13] Wharam MD, Hanfelt JJ, Tefft MC, et al. Radiation therapy for rhabdomyosarcoma: local failure risk for Clinical Group III patients on Intergroup Rhabdomyosarcoma Study II[J]. Int J Radiat Oncol Biol Phys, 1997, 38(4): 797-804. doi:10.1016/s0360-3016(97)00120-x.
[14] Lockney NA, Friedman DN, Wexler LH, et al. Late toxicities of intensity-modulated radiation therapy for head and neck rhabdomyosarcoma[J]. Pediatr Blood Cancer, 2016, 63(9): 1608-1614. doi:10.1002/pbc.26061.
[15] Hu JW, Liu SL, Qiu JH. Embryonal rhabdomyosarcoma of the middle ear[J]. Otolaryngol Head Neck Surg, 2002, 126(6): 690-692. doi:10.1067/mhn.2002.123922.
[16] Linabery AM, Ross JA. Childhood and adolescent cancer survival in the US by race and ethnicity for the diagnostic period 1975-1999[J]. Cancer, 2008, 113(9): 2575-2596. doi:10.1002/cncr.23866.
[17] Skapek SX, Ferrari A, Gupta AA, et al. Rhabdomyosarcoma[J]. Nat Rev Dis Primers, 2019, 5(1): 1-19. doi:10.1038/s41572-018-0051-2.
[18] Hibbitts E, Chi YY, Hawkins DS, et al. Refinement of risk stratification for childhood rhabdomyosarcoma using FOXO1 fusion status in addition to established clinical outcome predictors: a report from the Children's Oncology Group[J]. Cancer Med, 2019, 8(14): 6437-6448. doi:10.1002/cam4.2504.
[19] 中华医学会病理学分会儿科病理学组, 中国抗癌协会小儿肿瘤专业委员会病理学组, 福棠儿童医学发展研究中心病理专业委员会. 儿童横纹肌肉瘤病理诊断规范化专家共识[J]. 中华病理学杂志, 2021, 50(10): 1110-1115. doi:10.3760/cma.j.cn112151-20210112-00033. Consensus on the diagnosis of pediatric rhabdomyosarcoma[J]. Chinese Journal of Pathology, 2021, 50(10): 1110-1115. doi:10.3760/cma.j.cn112151-20210112-00033.
[20] Malempati S, Hawkins DS. Rhabdomyosarcoma: review of the Children's Oncology Group(COG)Soft-Tissue Sarcoma Committee experience and rationale for current COG studies[J]. Pediatr Blood Cancer, 2012, 59(1): 5-10. doi:10.1002/pbc.24118.
[21] 徐红艳, 黄慧, 杨文萍. 小儿耳鼻部胚胎性横纹肌肉瘤2例并文献复习[J]. 临床与实验病理学杂志, 2013, 29(4): 445-446. doi:10.13315/j.cnki.cjcep.2013.04.017.
[1] YANG Yang, WANG Xiaoxu, ZHANG Jie. Progress on diagnosis and treatment of middle ear cholesteatoma in children [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2022, 36(1): 1-6.
[2] NI Kun, SUN Shibing, LI Xiaoyan. Application of 1 000 Hz acoustic immittance in hearing screenings of high-risk newborns [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2022, 36(1): 43-47.
[3] HUANG Guiliang, SUN Changzhi, LUO Renzhong, CHEN Yanqiu, LIU Shaofeng, XU Jiajian. Clinical application analysis of endoscope in the operation of infantile tongue base cyst [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2022, 36(1): 100-105.
[4] WAN Yining, ZHANG Dejun, FU Zeming, GUO Fang, GUO Yingyuan, GUAN Guofang. Diffusion-weighted imaging for accurate diagnosis of congenital middle ear cholesteatoma and JOS staging [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2021, 35(6): 65-69.
[5] FAN HuijuanOverview,ZHANG HailiGuidance. Advancements of HIF-1α and VEGF in middle ear cholesteatoma [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2021, 35(5): 93-97.
[6] CHEN Kun, LI Lei, MENG Guozhen, YANG Jun, HOU Dongming. Diagnosis and nasal endoscopic surgery of congenital basal meningoencephaloceles in infants [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2021, 35(1): 77-81.
[7] Newborns and infants with hearing loss often suffer from vestibular dysfunction, which may aggravate with the gradual deterioration of hearing, resulting in delayed motor development or motor dysplasia. The motor development of newborns and infants depends on the function of the vestibular organs; thus, early vestibular function evaluation has positive prognostic significance. However, newborns and infants lack the language skills to fully express their symptoms. Furthermore, a series of complex vestibular function tests may cause them discomfort, such as vertigo and nausea, and the equipment and environment often scare them, which make vestibular assessment very challenging. Additionally, the structure and function of the vestibular, visual, and proprioceptive systems gradually mature with growth and development. Therefore, the clinical data of vestibular quantitative tests in different age groups cannot be compared with the standard reference range in adults. Hence, not all types of vestibular tests are necessary or appropriate in newborns and infants and should be adjusted according to the stage of development. In this study, we performed a literature review with focus on the common vestibular function tests according to the age and developmental stage of newborns and infants.. Update on the development and evaluation of vestibular function in newborns and Infants [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2020, 34(5): 82-88.
[8] QING Xiaoyan, XU YiquanOverview, LI ChaoGuidance. Advances in molecular mechanisms of anaplastic thyroid cancer [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2020, 34(3): 26-31.
[9] Yuxin TIAN,Jingjing WANG,Hui WANG,Yaqin WU,Haibo SHI,Zhengnong CHEN. Surgical treatment of iatrogenic facial paralysis [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2020, 34(1): 20-24.
[10] Zhao HAN, Juan DING. Physiology and pathology review of the middle ear muscles [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2019, 33(5): 6-10.
[11] NI Jing, LEI Fei, BAI Dan, ZHOU Yun, MA Jianmei, HUANG Wei, WANG Chunyang. Expression of immunological markers in middle ear effusion in children with secretory otitis media [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2019, 33(4): 56-59.
[12] YUAN Haiqin, CHEN Miaohong, ZHAO Jinfeng, ZHANG Guoming. Refractive study of children with spontaneous regression of retinopathy of prematurity [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2019, 33(4): 115-118.
[13] Fangzhou QIAN,Xiaobo HUANG,Qinjun WEI,Zhibin CHEN. Temporal and spatial expression of the deafness-causing gene EYA4 during the embryonic development of zebrafish [J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2019, 33(3): 49-55.
[14] MENG Wei, YU Zhenkun, ZHENG Yali, LI Guangfei, LIANG Longjun, WANG Xiaomin. Simulation of the biomechanical effects of deaf appendage implantation in mastoid cortical bone [J]. J Otolaryngol Ophthalmol Shandong Univ, 2018, 32(5): 27-30.
[15] MENG Lixin, LI Jian, WEI Shanwen. Insertion of microtubules in tympanic membrane for the treatment of middle ear atelectasis [J]. J Otolaryngol Ophthalmol Shandong Univ, 2018, 32(5): 61-65.
Viewed
Full text


Abstract

Cited
  Shared   
  Discussed   
No Suggested Reading articles found!
Website Copyright: Editorial Office of Journal of Otolaryngology and Ophthalmology of Shandong University
Address: No.27 Shanda South Road, Jinan, Shandong, China. Post code: 250100 Tel: +86-0531-88366912 Email: ebhxb@sdu.edu.cn Supported by Beijing Magtech Co.Ltd