PURPOSE:To investigate the epidemiology and clinicopathological management for ocular sebaceous carcinoma (OSC) in the UK. METHODS:Observational prospective cohort study of patients with newly-diagnosed OSC. The British Ophthalmological Surveillance Unit captured incident cases of OSC between 2008 and 2010. Incident and 6-month follow-up questionnaires from reporting ophthalmologists captured OSC demographic and clinical data. RESULTS:Data were available on 51 patients with unilateral OSC (response rate 85%). The UK estimated annual incidence was 0.41 cases per million population (95% CI 0.31 to 0.54). Median age was 70 years (SD 14, range 28-98) with 57% women. OSC location was upper lid (54%), lower lid (20%), multicentric (14%) and caruncle (12%). Most common misdiagnoses included chalazion (42%), basal cell carcinoma (30%) and blepharoconjunctivitis (16%), with median delay in diagnosis of 10 months (SD 9, range 0.5-36). Specialist ophthalmic pathologists performed diagnostics in 62%, with pagetoid/intraepithelial spread present in 39%. Misdiagnosis of chalazion (p=0.019) and pagetoid tumour spread (p=0.016) was associated with a significant diagnostic delay (one-way ANOVA/R(2)). Primary surgical management involved excision with reconstruction (49%), primary exenteration (10%) and Mohs surgery (8%). There were three deaths (out of 51) during the study period; one patient died of OSC-related disease and the other two due to other causes. CONCLUSIONS:This population-based prospective study confirms OSC as a rare cancer in the UK. Masquerade syndromes result in significant diagnostic delays and increase the risk of pagetoid tumour spread. There is considerable UK variation in pathological and surgical management, and ocular reconstruction and radical surgery is often required for OSC due to delayed presentation.

译文

目的:探讨英国眼皮脂腺癌(OSC)的流行病学和临床病理管理。
方法:对新诊断为OSC的患者进行观察性前瞻性队列研究。英国眼科监视部门捕获了2008年至2010年之间发生OSC的事件。来自报告眼科医师的事件和6个月的后续调查表捕获了OSC的人口统计和临床数据。
结果:有51例单侧OSC患者的数据(有效率85%)。英国估计的年发病率为每百万人口0.41例(95%CI为0.31至0.54)。中位年龄为70岁(SD 14,范围28-98),其中女性占57%。 OSC位置是上盖(54%),下盖(20%),多中心(14%)和and形(12%)。最常见的误诊包括睑板裂(42%),基底细胞癌(30%)和睑结膜炎(16%),中位诊断时间延迟10个月(SD 9,范围0.5-36)。专门的眼科病理学家对62%的患者进行了诊断,其中39%的患者出现了页面状/上皮内扩散。龟头孢子虫(p = 0.019)和页面状肿瘤扩散(p = 0.016)的误诊与显着的诊断延迟有关(单向ANOVA / R(2))。主要的外科手术管理包括切除术,重建术(49%),初次切除术(10%)和莫氏手术(8%)。在研究期间,有3例死亡(共51例)。一名患者死于OSC相关疾病,另一例死于其他原因。
结论:这项基于人群的前瞻性研究证实了OSC在英国是一种罕见的癌症。假面舞会综合症会导致严重的诊断延迟,并增加页面状肿瘤扩散的风险。英国在病理和手术管理上存在很大差异,由于延迟出现,OSC经常需要眼球重建和根治性手术。

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