Department of Pathology, Tata Memorial Hospital, Mumbai, India.
Arch Pathol Lab Med. 2011 Aug;135(8):1017-23. doi: 10.5858/2010-0311-OAR2.
Advanced cases of retinoblastoma are treated with chemoreduction followed by enucleation. Further adjuvant therapy is recommended in patients with known pathologic risk factors (PRFs).
To determine the PRFs in enucleated specimens after chemoreduction and their association for adverse events of recurrence, metastasis, or death.
This was a retrospective study of 77 enucleation specimens from patients treated between January 2000 and September 2008 with prior chemoreduction that were accessioned in the pathology department of a tertiary referral cancer center with an average follow-up of 24 months. Various PRFs were noted and their association with the development of an adverse event was recorded.
Of 77 patients, (male to female ratio, 51∶26), the incidence of overall PRF was 51.9%, and retrolaminar optic nerve invasion (32.5%), optic nerve cut margin (12.9%), massive choroidal invasion (26%), scleral invasion (23.4%), vitreous seedings (44.2%), and anterior segment invasion (20.8%). Undifferentiated tumor (>60%) was seen in 60.3% of cases (41 of 68 patients with differentiation available). Adverse event occurred in 18 of 72 patients with available follow-up (25%). Retrolaminar optic nerve invasion, optic nerve cut margin involvement, and scleral invasion were independent prognostic factors predicting the occurrence of an adverse event. Undifferentiated tumor (>60%) was a significant risk factor in univariate analysis, which is the unique feature in this study.
Classic PRF with the addition of a predominant presence from the undifferentiated component were associated with adverse outcomes in retinoblastoma treated with anterior chemotherapy. The latter may represent chemoresistant clones and more intensive adjuvant chemotherapy may be warranted in these patients.
晚期视网膜母细胞瘤患者采用化疗后行眼球摘除术治疗。已知存在病理危险因素（PRFs）的患者推荐进一步辅助治疗。
确定化疗后眼球摘除标本中的 PRFs 及其与复发、转移或死亡不良事件的相关性。
这是一项回顾性研究，纳入了 2000 年 1 月至 2008 年 9 月期间在一家三级转诊癌症中心接受化疗后行眼球摘除术的 77 例患者的 77 例眼球摘除标本，这些患者的病理标本均被病理科收录，平均随访时间为 24 个月。记录了各种 PRFs，并记录了它们与不良事件发展的相关性。
在 77 例患者中（男女比例为 51∶26），整体 PRF 发生率为 51.9%，视网膜后神经鞘侵犯（32.5%）、视神经切缘累及（12.9%）、广泛脉络膜侵犯（26%）、巩膜侵犯（23.4%）、玻璃体播散（44.2%）和前段侵犯（20.8%）。在可进行分化评估的 68 例患者中，有 60.3%（41 例）可见未分化肿瘤（>60%）。在有随访资料的 72 例患者中，有 18 例（25%）发生不良事件。视网膜后神经鞘侵犯、视神经切缘累及和巩膜侵犯是预测不良事件发生的独立预后因素。未分化肿瘤（>60%）在单因素分析中是一个显著的危险因素，这是本研究的一个独特特征。
在接受前化疗的视网膜母细胞瘤患者中，经典 PRFs 加上未分化成分的主要存在与不良结局相关。后者可能代表化疗耐药克隆，这些患者可能需要更强化的辅助化疗。
