Puthussery Jose Cijin, Wagner William, Singh Arun D
Cleveland Clinic, Cleveland, OH, USA.
Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA.
Ocul Oncol Pathol. 2025 Apr;11(1):37-45. doi: 10.1159/000543040. Epub 2024 Dec 9.
The aim of the study was to describe clinical features of lipid dominant toxic tumor syndrome (TTS) and report outcomes following use of intravitreal steroids.
Records of 13 patients who had lipid dominant TTS following treatment of choroidal melanoma with episcleral plaque brachytherapy (EPB) were retrospectively reviewed. Resolution of lipid exudates, subfoveal subretinal fluid, cystoid macular edema (CME), exudative detachment were main outcome measures.
Of the 13 patients who developed lipid dominant TTS, 11 (85%) had medium-sized melanomas, and 2 (15%) small-sized melanomas. The average time to onset following EPB was 22 months (range 3-48 months). Seven patients (54%) were noted to have dyslipidemia. The baseline visual acuity at the time of diagnosis of TTS was 50 ETDRS letters (range 10-85). Ophthalmic characteristics were lipid exudates centered around the tumor base in 13 (100%) patients, subfoveal subretinal fluid in 4 (31%) patients, and CME in 2 (15%) patients. Exudative detachment was absent in all (100%) patients. Regressed melanoma was present in all (100%) patients. Eight (62%) patients were treated with intravitreal steroids (4 mg triamcinolone), while 5 patients (38%) were observed. The response to intravitreal steroids was noted in 7(88%) of the treated patients, with the average time to response being 1.9 months. Features characterizing a positive response were reduction in lipid exudates centered around the tumor base (100%), reduction in subfoveal subretinal fluid (100%), and reduction in CME (50%). Cataract development was seen in 10 (83%) and ocular hypertension in 3 patients (23%). Proliferative radiation retinopathy developed in 2 (15%) patients, neovascular glaucoma developed in 1(8%) while no patients required enucleation.
The lipid dominant TTS centered around the tumor base that occurs in a radiation responsive tumor could be considered a chronic variant in the spectrum of TTS. Intravitreal steroids in selected cases reverse the course of this variant, stabilizing or improving the vision and avoiding enucleation. Our observations would need to be verified through a larger prospective study.
本研究旨在描述脂质为主型毒性肿瘤综合征（TTS）的临床特征，并报告玻璃体内注射类固醇后的治疗结果。
回顾性分析13例巩膜外斑块近距离放射治疗（EPB）脉络膜黑色素瘤后发生脂质为主型TTS患者的病历。脂质渗出物消退、黄斑中心凹下视网膜下液、黄斑囊样水肿（CME）、渗出性视网膜脱离的消退情况为主要观察指标。
13例发生脂质为主型TTS的患者中，11例（85%）为中等大小黑色素瘤，2例（15%）为小尺寸黑色素瘤。EPB后平均发病时间为22个月（范围3 - 48个月）。7例（54%）患者存在血脂异常。TTS诊断时的基线视力为50 ETDRS字母（范围10 - 85）。眼部特征方面，13例（100%）患者的脂质渗出物围绕肿瘤基底，4例（31%）患者有黄斑中心凹下视网膜下液，2例（15%）患者有CME。所有（100%）患者均无渗出性视网膜脱离。所有（100%）患者的黑色素瘤均有消退。8例（62%）患者接受玻璃体内类固醇治疗（4 mg曲安奈德），5例（38%）患者进行观察。7例（88%）接受治疗的患者对玻璃体内类固醇有反应，平均反应时间为1.9个月。阳性反应的特征包括围绕肿瘤基底的脂质渗出物减少（100%）、黄斑中心凹下视网膜下液减少（100%）以及CME减少（50%）。10例（83%）患者出现白内障，3例（23%）患者出现高眼压。2例（15%）患者发生增殖性放射性视网膜病变，1例（8%）患者发生新生血管性青光眼，无患者需要眼球摘除。
发生在对放射治疗有反应的肿瘤中的、以肿瘤基底为中心的脂质为主型TTS可被视为TTS谱系中的一种慢性变异型。在部分病例中，玻璃体内注射类固醇可逆转此变异型病程，稳定或改善视力并避免眼球摘除。我们的观察结果需要通过更大规模的前瞻性研究加以验证。
