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声门上区骨外尤文肉瘤:1例罕见病例报告。

Extraskeletal Ewing's sarcoma of supraglottis: A rare case report.

作者信息

Poojari Mayadevi, Madabhavi Irappa, Desai Sharad

机构信息

Consultant Radiation Oncology, Department of Radiation Oncology, Mahatma Gandhi Cancer Hospital, Miraj, Maharashtra, India.

Consultant Medical and Pediatric Oncologist and Hematologit, J N Medical College and KLE Academy of Higher Education and Research Belagavi, Kerudi Cancer Hospital, Bagalkot and Nanjappa Hospital, Davanagere, Karnataka, India..

出版信息

Oral Oncol. 2024 May;152:106789. doi: 10.1016/j.oraloncology.2024.106789. Epub 2024 Apr 5.

Abstract

Ewing's Sarcoma family of tumors is a group of small round tumor cells. Ewing's sarcoma majority occurs in bone, accounts about 10 % of primary bone tumors. Extraskeletal Ewing's sarcoma (ESS) is unusual and commonly seen in trunk, paravertebral, and chest wall region. It is rarely seen in head and neck region, accounting to 2-3 %. In head and neck region, ESS is seen in nasal or oral cavities, sinuses. EES originating in the larynx is very rare. Here, we report a 22 years old female having the complaints of change in voice and noisy breathing who was diagnosed as a case of EES of supraglottis. As the disease progressed during the time of diagnosis, she had to undergo emergency tracheostomy. The disease was inoperable so she received neoadjuvant chemotherapy followed by radiation followed by adjuvant chemotherapy. At present she is symptomatically better. The aim of this report is to put forward the rare site of Ewing's Sarcoma and highlighting the early diagnosis in suspected case with IHC, providing effective multimodality treatment.

摘要

尤因肉瘤家族性肿瘤是一组小圆形肿瘤细胞。尤因肉瘤大多发生于骨骼,约占原发性骨肿瘤的10%。骨外尤因肉瘤(ESS)较为少见,常见于躯干、椎旁和胸壁区域。在头颈部区域罕见,占2% - 3%。在头颈部区域,ESS可见于鼻腔、口腔或鼻窦。起源于喉的尤因肉瘤非常罕见。在此,我们报告一例22岁女性,因声音改变和呼吸嘈杂前来就诊,被诊断为声门上区尤因肉瘤。在诊断时病情进展,她不得不接受紧急气管切开术。该疾病无法手术切除,因此她接受了新辅助化疗,随后进行放疗,再进行辅助化疗。目前她的症状有所改善。本报告的目的是提出尤因肉瘤的罕见发病部位,并强调对疑似病例通过免疫组化进行早期诊断,提供有效的多模式治疗。

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