额颞部头皮原发性上皮样肉瘤:罕见病例报告及最新文献综述

G. D. Adhikari, Sandip Kumar Mandal, Suman Gnawali, Gyan B  Shrestha, Nitu Sharma, Ajay Kumar Yadav
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摘要

上皮样肉瘤(ES)是一种非常罕见的侵袭性间叶肉瘤亚型,在软组织肉瘤(STS)中所占比例不到 1%。根据部位的起源,ES 可分为两种类型:远端型上皮样肉瘤和近端型上皮样肉瘤。ES 的临床诊断主要通过组织病理学检查和免疫组化来确定。 根治性切除手术是上皮样肉瘤的最佳治疗方案。根据 TNM 分期,治疗方案会有所不同,有的需要手术,有的需要放疗和化疗。在本报告中,我们介绍了一名 36 岁的男性,他的左额颞部头皮溃疡不愈合,并延伸至左眼眶。组织病理学证实为上皮样肉瘤。头颈部磁共振成像(MRI)显示,左额颞部头皮有一肿块,大小为 6.7 X 5.7 X 2.6 厘米,延伸至颧部和左眼眶 D/D恶性肿块/肉瘤,伴有双侧(B/L)鼻窦炎。之后,患者于 2022 年 9 月 11 日接受了大范围局部切除(WLE)手术。由于疾病局部进展,患者在 2022 年 9 月 22 日至 11 月 10 日期间接受了辅助放射治疗(RT),放射剂量为 6000 cGy,共分 30 次(#),每次(#)200 cGy。患者被要求在 RT 结束后 6 周进行随访。手术 6 周后,患者接受了磁共振成像检查,报告显示检查结果显示正常。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Primary Epithelioid Sarcoma of Frontotemporal Scalp: a Rare Case Report with Recent Literature Review
Epithelioid sarcoma (ES) is a very rare and aggressive mesenchymal sarcoma subtype which represents less than 1% of soft tissue sarcomas (STS). According to the origin of the site, there are two types of ES: distal-type epithelioid sarcoma and proximal-type epithelioid sarcoma. The clinical diagnosis of ES mainly is confirmed by histopathology examination followed by immunohistochemistry.  Radical excisional surgery is the best treatment option for epithelioid sarcoma. According to TNM staging, the treatment option will vary from surgery to surgery accompanied with radiotherapy and chemotherapy. Here in, we presented a 36-year-old man had non-healing ulcer in left frontotemporal scalp extending to involve left orbit. Histopathological confirmed epithelioid sarcoma. Magnetic Resonance Imaging (MRI) of Head and Neck revealed that there was mass in left frontotemporal scalp measuring 6.7 X 5.7 X 2.6 cm extending to zygomatic region and left orbit D/D malignant mass/sarcoma with bilateral (B/L) sinusitis. After that patient undergone for Wide Local excision (WLE) surgery on 11th September 2022. Adjuvant Radiation Therapy (RT) 6000 cGy radiation dose in 30 fractions (#) which was 200 cGy per fraction (#) were given during 22nd September to 10th November 2022 due to local advancement of disease. Patient was asked to follow up after 6 weeks after completion of RT. After 6 weeks of surgery, the patient was undergone for MRI and report revealed normal study.
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