Lam D S, Li C K, Cheng L L, Teo J G, Chik K W, Kwan W H, Ng J S, Poon W W, Yuen P M
Department of Ophthalmology and Visual Sciences, Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, N.T.
Eye (Lond). 1999;13 ( Pt 1):38-42. doi: 10.1038/eye.1999.8.
Primary orbital Ewing's sarcoma is a very rare condition. Since the first case was reported in 1950, only 7 other cases have been reported in the English literature. Herein we describe the ninth, the only bilateral and the youngest case of primary orbital Ewing's sarcoma occurring in a 2-year-old boy, who presented to us with bilateral painless proptosis. Tissue biopsy of the tumour was obtained through the nasal sinus. Immunohistological studies of the biopsy tissue confirmed the diagnosis of Ewing's sarcoma. No distant site of the tumour was found so this was considered a primary orbital tumour. Combined chemotherapy and radiotherapy without surgical resection achieved an encouraging result in that the patient has remained in remission for 30 months after completion of treatment. The successful use of combined chemotherapy and radiotherapy, without surgery, adds further support to evidence that surgical excision may be avoided in selected cases of primary orbital Ewing's sarcoma.
原发性眼眶尤文肉瘤是一种非常罕见的疾病。自1950年首例病例报告以来，英文文献中仅另有7例报告。在此，我们描述第9例原发性眼眶尤文肉瘤病例，这是唯一的双侧发病且最年轻的病例，发生在一名2岁男孩身上，他因双侧无痛性眼球突出前来就诊。肿瘤组织活检通过鼻窦获取。活检组织的免疫组织学研究确诊为尤文肉瘤。未发现肿瘤远处转移，因此被认为是原发性眼眶肿瘤。联合化疗和放疗而未进行手术切除取得了令人鼓舞的结果，即患者在完成治疗后已缓解30个月。联合化疗和放疗而非手术的成功应用，进一步支持了在原发性眼眶尤文肉瘤的某些病例中可避免手术切除的证据。
