Dutton J J, Rose J G, DeBacker C M, Gayre G
The Department of Ophthalmology, Duke University Eye Center, Durham, North Carolina, USA.
Ophthalmic Plast Reconstr Surg. 2000 Jul;16(4):292-300. doi: 10.1097/00002341-200007000-00008.
Ewing's tumor is a primary tumor of bone in childhood that only rarely involves the orbit. Most such cases are metastatic from distant sites. This tumor may be confused with other small round cell malignancies of childhood, and immunohistochemical studies are essential in making the diagnosis.
We present two cases of Ewing's tumor of the orbit. One was in a 22-year-old boy with an occult primary tumor in the frontal bone that became symptomatic after forehead trauma. The other example was in a 7-year-old boy with a known Ewing's primary of the clavicle. The clinical manifestations and diagnostic criteria are discussed.
In most cases with orbital involvement, ophthalmic symptoms consist of proptosis, pain, and occasionally visual loss and motility restriction. The diagnosis is typically unsuspected before histologic evaluation. Electron microscopic and immunohistochemical analyses are essential in making the diagnosis and are necessary for all such small round cell tumors.
Ewing's sarcoma is a rare orbital tumor of bone mainly affecting children. Local treatment relying on surgical extirpation and radiotherapy alone has proven inadequate, with 5-year survival rates of <10%. The addition of chemotherapy has improved survival rates significantly to approximately 50%.
尤因肉瘤是儿童期的一种原发性骨肿瘤，很少累及眼眶。大多数此类病例是远处转移而来。该肿瘤可能与儿童期其他小圆形细胞恶性肿瘤相混淆，免疫组化研究对做出诊断至关重要。
我们报告两例眼眶尤因肉瘤病例。一例为一名22岁男孩，额骨有隐匿性原发肿瘤，前额外伤后出现症状。另一例是一名7岁男孩，已知锁骨有尤因肉瘤原发灶。讨论了临床表现和诊断标准。
在大多数累及眼眶的病例中，眼部症状包括眼球突出、疼痛，偶尔还有视力丧失和眼球运动受限。在组织学评估之前，通常无法怀疑诊断。电子显微镜和免疫组化分析对做出诊断至关重要，对所有此类小圆形细胞肿瘤都是必要的。
尤因肉瘤是一种罕见的主要影响儿童的眼眶骨肿瘤。仅依靠手术切除和放疗的局部治疗已被证明是不够的，5年生存率<10%。化疗的加入显著提高了生存率，约为50%。
