Chandra Kiran, Raval Vishal, Reddy P Vijay Anand, Kaliki Swathi
Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, India.
J Vitreoretin Dis. 2022 Aug 4;6(5):367-373. doi: 10.1177/24741264221107757. eCollection 2022 Sep-Oct.
To describe the clinical features and treatment outcomes in eyes with primary macular retinoblastoma (RB). Patients with primary macular RB were analyzed. Of 41 patients (47 eyes), 20 (49%) were boys and 21 (51%) were girls. The mean age at diagnosis was 16 months (range, 1-60 months). The RB was bilateral in 6 patients (15%). At presentation, the macula was completely covered with the tumor in 22 eyes (47%) and partially covered with the fovea spared in 13 eyes (28%) and the fovea involved 12 eyes (25%). Based on the International Classification of Intraocular Retinoblastoma, 25 (53%), 15 (32%), and 7 (15%) of tumors were in Group B, Group C, and Group D, respectively. The tumor had exophytic features in 36 eyes (77%). The mean tumor basal diameter was 10.0 mm and the mean thickness, 5.6 mm. Associated features included subretinal seeds (10 eyes; 21%) and surrounding subretinal fluid (16 eyes; 34%). Forty-three eyes (92%) were treated with intravenous chemotherapy, 2 (4%) with intra-arterial chemotherapy, and 2 (4%) with transpupillary thermotherapy. Local tumor control was achieved in 45 eyes (96%), with 33 eyes (70%) showing a type III regression pattern. Over a mean follow-up of 23 months (range, 3-48 months), the macular tumor recurred in 5 eyes (11%), the globe was salvaged in all eyes with associated foveal atrophy (36 eyes; 77%), and 1 patient (2%) died. Macular RB has a good prognosis for globe salvage while vision salvage might be compromised as a result of associated foveal atrophy.
描述原发性黄斑视网膜母细胞瘤（RB）患眼的临床特征及治疗结果。对原发性黄斑RB患者进行分析。41例患者（47只眼）中，20例（49%）为男孩，21例（51%）为女孩。诊断时的平均年龄为16个月（范围1 - 60个月）。6例（15%）患者的RB为双侧性。就诊时，22只眼（47%）的黄斑完全被肿瘤覆盖，13只眼（28%）的黄斑部分被肿瘤覆盖，中央凹未受累，12只眼（25%）的中央凹受累。根据国际眼内视网膜母细胞瘤分类，分别有25只眼（53%）、15只眼（32%）和7只眼（15%）的肿瘤属于B组、C组和D组。36只眼（77%）的肿瘤具有外生性特征。肿瘤基底平均直径为10.0 mm，平均厚度为5.6 mm。相关特征包括视网膜下播散灶（10只眼；21%）和周围视网膜下液（16只眼；34%）。43只眼（92%）接受静脉化疗，2只眼（4%）接受动脉内化疗，2只眼（4%）接受经瞳孔温热疗法。45只眼（96%）实现了局部肿瘤控制，33只眼（70%）呈现III型消退模式。平均随访23个月（范围3 - 48个月），5只眼（11%）的黄斑肿瘤复发，所有伴有中央凹萎缩的眼（36只眼；77%）均保住了眼球，1例患者（2%）死亡。黄斑RB在保眼方面预后良好，但由于伴有中央凹萎缩，视力保留可能会受到影响。
