Günalp I, Gündüz K, Arslan Y
Ocular Oncology Department, Faculty of Medicine, University of Ankara, Turkey.
Jpn J Ophthalmol. 1996;40(1):95-102.
The treatment modalities and prognosis of 636 retinoblastoma (RB) cases diagnosed and treated in our specialist center between 1963 and 1994 were evaluated. Patient age ranged from 20 days to 16 years, the mean age being 2.2 years (26.4 months). Of the 636 cases, 441 were unilateral and 195 were bilateral. Enucleation was the most frequent treatment employed in unilateral RB patients (412 cases). Follow-up treatment included exenteration (48 cases), radiotherapy (154 cases) and chemotherapy (108 cases) for cases with optic nerve invasion and/or orbital recurrence following enucleation. Seventeen cases displayed massive proptosis, ocular damage and blindness at initial presentation and underwent exenteration as the initial treatment. Two cases were subjected to external beam radiotherapy without invasive surgical procedures. Ten cases regressed spontaneously without treatment. For bilateral cases, the most frequent treatment used was enucleation for one eye and radiotherapy for the other (132 cases). Adjuvant treatment included exenteration (9 cases) and chemotherapy (50 cases) depending on orbital recurrence and/or systemic metastasis. Spontaneous bilateral regression was noted in one case. Six cases underwent bilateral external beam radiotherapy without surgery. One eye of the remaining 56 bilateral cases underwent enucleation. The treatment for the contralateral eyes included cryotherapy in 14 cases, enucleation in 11 cases, Cobalt plaque (Co plaque) therapy in 10 cases, photocoagulation in 6 cases and exenteration in one case. No treatment was undertaken in the contralateral eyes of 14 cases. Secondary treatment modalities employed in these 56 bilateral cases were radiotherapy (11 cases), chemotherapy (8 cases), Co plaque (8 cases) and exenteration (5 cases). Treatment complications were detected in 25 cases followed for at least 18 months. Eighteen cases had radiation cataracts and 6 of these 18 patients underwent intraocular lens implantation. Post-radiation orbital malignancy (osteosarcoma) was noted in two cases aged 14 and 15 years. Phthisis bulbi was observed in three cases and radiation keratitis in two cases. The overall survival rate was 82.2% after a mean follow-up of 5 years. The survival rate of unilateral cases was 82.8% and that of bilateral cases was 81.1% at 5 years.
对1963年至1994年间在我们专业中心诊断并接受治疗的636例视网膜母细胞瘤（RB）病例的治疗方式和预后进行了评估。患者年龄从20天至16岁不等，平均年龄为2.2岁（26.4个月）。在这636例病例中，441例为单侧发病，195例为双侧发病。眼球摘除术是单侧RB患者最常用的治疗方法（412例）。后续治疗包括对眼球摘除术后出现视神经侵犯和/或眼眶复发的病例进行眶内容剜除术（48例）、放射治疗（154例）和化学治疗（108例）。17例患者初诊时表现为眼球明显突出、眼组织破坏和失明，初始治疗为眶内容剜除术。2例患者未进行侵入性手术，仅接受了外照射放疗。10例患者未经治疗自行消退。对于双侧病例，最常用的治疗方法是一眼进行眼球摘除术，另一眼进行放射治疗（132例）。辅助治疗包括根据眼眶复发和/或全身转移情况进行眶内容剜除术（9例）和化学治疗（50例）。1例患者出现双侧自发消退。6例患者未进行手术，仅接受了双侧外照射放疗。其余56例双侧病例中，1只眼进行了眼球摘除术。对侧眼的治疗包括冷冻疗法14例、眼球摘除术11例、钴板（Co板）治疗10例、光凝治疗6例和眶内容剜除术1例。14例患者的对侧眼未进行治疗。在这56例双侧病例中采用的二次治疗方式包括放射治疗（11例）、化学治疗（8例）、Co板治疗（8例）和眶内容剜除术（5例）。对至少随访18个月的25例患者检测到治疗并发症。18例患者出现放射性白内障，其中6例患者接受了人工晶状体植入术。2例年龄分别为14岁和15岁的患者出现放射后眼眶恶性肿瘤（骨肉瘤）。3例患者出现眼球痨，2例患者出现放射性角膜炎。平均随访5年后，总生存率为82.2%。5年时，单侧病例的生存率为82.8%，双侧病例的生存率为81.1%。
