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儿童胚胎性横纹肌肉瘤4例治疗病例系列

Case Series on Four Treated Cases of Embryonal Rhabdomyosarcoma in Children.

作者信息

Gupta Amit, Sasidharan Jaseetha, Bhargava Aditya

机构信息

Department of ENT, ESI PGIMSR, Basaidarapur, Guru Gobind Singh Indraprastha University, New Delhi, India.

出版信息

Indian J Otolaryngol Head Neck Surg. 2020 Jun;72(2):214-220. doi: 10.1007/s12070-019-01767-y. Epub 2019 Nov 26.

Abstract

We report four cases of embryonal rhabdomyosarcoma treated successfully with multimodality therapy which included surgery, chemotherapy and radiotherapy. Our first case was a 2 year old who admitted with the chief complaint of swelling of the upper lip. Biopsy was taken, which showed embryonal rhabdomyosarcoma. It was followed by a CT scan (face + neck), MRI face, USG abdomen and bone scan of the patient. Pre operatively chemotherapy was given. After completion of chemotherapy regimen, wide local excision of the lesion along with a reverse fan flap and bilateral modified radical neck dissection type 3 was done. This was followed by post operative chemotherapy and radiotherapy. There was no recurrence at 6 months post operatively. The second patient was 6 years old with similar complaints, after undertaking all the investigations as in the previous case, patient was given neoadjuvant chemotherapy following which wide local excision of the lesion along with reconstruction with the help of bilateral nasolabial flap was done. This was followed by post operative chemotherapy. No recurrence was present 6 months post operatively. The third patient was a 17 year old male presenting with complaints of left sided nasal obstruction and left nasal swelling. Examination revealed left sided nasal mass which on biopsy turned out to be embryonal rhabdomyosarcoma. The patient underwent surgery followed by chemoradiation. At 6 months the patient had no evidence of recurrence. The fourth patient was 16-year-old male who presented with complaints of right sided nasal swelling and nasal obstruction. Radiological investigation showed the tumor involving the right sinonasal cavity with extension to anterior cranial fossa and with neck nodes. Patient was given concurrent chemoradiation. Patient was followed up for 6 months when the patient presented with recurrence of disease with distant metastasis. Patient is currently on palliative chemotherapy. To summarise, embryonal rhabdomyosarcoma in young children when treated aggressively with surgery and chemotherapy has better prognosis than with chemo-radiation alone.

摘要

我们报告了4例采用多模式疗法成功治疗的胚胎性横纹肌肉瘤病例,该疗法包括手术、化疗和放疗。我们的首例病例是一名2岁儿童,主要症状为上唇肿胀。进行了活检,结果显示为胚胎性横纹肌肉瘤。随后对该患者进行了CT扫描(面部+颈部)、面部MRI、腹部超声检查和骨扫描。术前给予化疗。化疗方案完成后,对病变进行了广泛局部切除,同时进行了反向扇形皮瓣和双侧改良根治性颈清扫3型手术。随后进行了术后化疗和放疗。术后6个月无复发。第二例患者为6岁,有类似症状,在进行了与前一例相同的所有检查后,给予新辅助化疗,随后对病变进行了广泛局部切除,并借助双侧鼻唇瓣进行了重建。随后进行了术后化疗。术后6个月无复发。第三例患者是一名17岁男性,主诉左侧鼻塞和左侧鼻肿胀。检查发现左侧鼻腔肿物,活检结果为胚胎性横纹肌肉瘤。该患者接受了手术,随后进行了放化疗。6个月时患者无复发迹象。第四例患者是一名16岁男性,主诉右侧鼻肿胀和鼻塞。影像学检查显示肿瘤累及右侧鼻窦腔,延伸至前颅窝并伴有颈部淋巴结。患者接受了同步放化疗。对患者进行了6个月的随访,此时患者出现疾病复发并伴有远处转移。患者目前正在接受姑息化疗。总之,幼儿的胚胎性横纹肌肉瘤采用手术和化疗积极治疗时,预后比单纯放化疗更好。

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