【摘要】 目的：回顾1997/2007伊朗亚兹德地区的200例眼及附属器肿瘤的流行病学特征，确定肿瘤的人群分布及肿瘤位置。 方法：回顾观察性病例报道。我们回顾1997/2007医院组织病理学确诊为眼眶肿瘤的病例，分析200例连续病例的年龄分布、病理学、肿瘤来源及肿瘤位置。结果：患者200例（男111例，女89例），其中110例(55.0%)为良性肿瘤，86例 (43.0 %)为恶性，4例(2.0%)为转移性肿瘤。119 例 (59.5%)为眼睑肿瘤，21 例(10.5%)为眼眶，60例(30.0%)位于眼球。最常见的恶性肿瘤是基底细胞癌(BCC) (25.5%)、鳞状细胞癌(SCC) (6.0%)及黑素瘤(5.5%)。各种类型的痣(15.0%)、皮样囊肿(5.5%)、血管瘤(5.0%)是最常见的良性肿瘤。200例患者按年龄分为两组，18岁以上及18岁以下。18岁以下患者中，最常见的是良性肿瘤(90.1%)，恶性肿瘤及转移性肿瘤分别占8.4% 和1.5%。然而，18岁以上患者，常见的是恶性肿瘤(51.1%)，良性肿瘤占47.1%，转移性肿瘤占1.8%。结论：眼眶肿瘤的病理学特征与患者的年龄及肿瘤在眼及附属器的位置有关。在进行活检及肿瘤切除术之前，患者的发病年龄和肿瘤位置可成为我们制定治疗方案的前提，也是预后评估的重要信息。
【关键词】 眼眶肿瘤 恶性的 良性肿瘤 转移性肿瘤
INTRODUCTION
Retinoblastoma is the most common primary intraocular malignancy in children. A bimodal distribution of age at diagnosis has been described; 12 months of age for germline tumors and 1824 months of age for sporadic tumors[1]. The most common presentation of retinoblastoma is leukocoria, followed by strabismus. Rarely, a child can present later in life with ocular inflammation leading to a delay in diagnosis[2]. Diffuse infiltrating retinoblastoma accounts for approximately 1% to 2% of retinoblastoma cases[3]. We report a case of diffuse infiltrating retinoblastoma presenting as endophthalmitis in an 8yearold girl and comment on the histopathologic findings.
CASE REPORT
An 8yearold Chinese girl developed painless decreased vision in the right eye over the course of six days. She was otherwise healthy, no history of trauma or family history of retinoblastoma. Her visual acuity was recorded as hand motions at 10cm in the right eye and 20/20 in the left eye. Slitlamp examination of the right eye revealed the presence of multiple white deposits on the posterior capsule of the lens. Ophthalmoscopic examination showed white opacification of the entire vitreous, but no solid mass. Details of the fundus could not be visualized. Bscan ultrasonography demonstrated vitreous organization, stranding, and posterior vitreous separation, without evidence of a distinct mass or areas of calcification (Figure 1A). A clinical diagnosis of endogenous endophthalmitis with unknown primary source was made and vitrectomy was performed. During vitrectomy, no retinal mass, thickening or detachment was identified and silicone oil was infused prophylactically.
The silicone oil was removed 3 months later. A flat, slightly white membrane overlying the optic disc was identified and biopsied. Fellow eye examination was normal. On the following day, the vitreous cavity became opacified again and discrete nodular areas of calcification were noted on Bscan ultrasonography (Figure 1B). In addition, computed tomography (CT) demonstrated calcification at the pars plana without a discrete retina mass (Figure 1C). Histopathological analysis revealed very dense aggregations of small round cells, intermingled with poorly staining areas(Figure 2A).Immunohistochemical studies were performed and were variously positive: Synaptophysin(+) (Figure 2B), Cga(+), S100(+),Lca(), Mpo(), and Myogenin(). This staining pattern supported the diagnosis of retinoblastoma. The patients parents declined enucleation. Instead, the patient was referred to the oncology service for chemotherapy, which consisted of vinblastine sulfate, etoposide and carboplatin. At the 6 month followup visit, the patient was free of metastases.
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