A Rare Case of Ewing Sarcoma of the Left Ethmoid Sinus With Orbital Extension.

IF 2.3 3区医学 Q1 OTORHINOLARYNGOLOGY Head and Neck-Journal for the Sciences and Specialties of the Head and Neck Pub Date : 2024-11-11 DOI:10.1002/hed.27993

Xiaoxuan Chen, Kiran Abraham-Aggarwal, David I Kutler

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Abstract

Background: Ewing sarcoma (ES) of the ethmoid sinus with orbital involvement in an adult is very rare, with 16 reported cases in the literature. Immunohistochemical studies show small blue round cells positive for CD99 and fluorescence in situ hybridization (FISH) testing reveals positivity for the EWSR1 gene.

Methods: A 38-year-old male with a diagnosis of ES of the ethmoid sinus presented with left-sided periorbital pain and edema, rhinorrhea, and proptosis. The patient underwent neoadjuvant chemotherapy, surgical resection of the left skull base, and postoperative proton radiotherapy.

Results: The patient tolerated chemotherapy, surgical resection, and adjuvant proton radiotherapy well with resolution of proptosis, diplopia, and pain. Due to local recurrence, he is currently undergoing adjuvant chemotherapy.

Conclusion: Our findings provide insight on the clinical presentation and appropriate management of extraosseous ES, specifically in the ethmoid sinus in the adult population.

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左侧乙状窦尤文肉瘤伴眼眶扩展的罕见病例

背景：成人乙状窦埃文肉瘤（ES）累及眼眶的病例非常罕见，文献中仅有16例报道。免疫组化研究显示小的蓝色圆形细胞 CD99 阳性，荧光原位杂交（FISH）检测显示 EWSR1 基因阳性：一名 38 岁的男性患者被诊断为乙状窦 ES，并伴有左侧眶周疼痛和水肿、鼻出血和突眼。患者接受了新辅助化疗、左侧颅底手术切除和术后质子放疗：患者对化疗、手术切除和辅助质子放疗的耐受性良好，突眼、复视和疼痛均已缓解。由于局部复发，他目前正在接受辅助化疗：我们的研究结果为骨外ES，尤其是成人乙状窦骨外ES的临床表现和适当治疗提供了启示。

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来源期刊

Head and Neck-Journal for the Sciences and Specialties of the Head and Neck 医学-耳鼻喉科学

CiteScore

7.00

自引率

6.90%

发文量

278

审稿时长

1.6 months

期刊介绍： Head & Neck is an international multidisciplinary publication of original contributions concerning the diagnosis and management of diseases of the head and neck. This area involves the overlapping interests and expertise of several surgical and medical specialties, including general surgery, neurosurgery, otolaryngology, plastic surgery, oral surgery, dermatology, ophthalmology, pathology, radiotherapy, medical oncology, and the corresponding basic sciences.