Ocular Pathology Services, New Delhi, India.
Clin Exp Ophthalmol. 2011 Mar;39(2):105-10. doi: 10.1111/j.1442-9071.2010.02426.x. Epub 2011 Feb 22.
Phthisis bulbi is a relatively uncommon and atypical clinical presentation of retinoblastoma.
Retrospective study conducted at a tertiary care hospital.
Eighteen consecutive retinoblastoma patients with primary phthisis bulbi.
Retrospective analysis of clinical, imaging and histopathological features of all retinoblastoma patients with primary phthisis bulbi, treated at our centre between January 2005 and December 2009.
Clinical and histopathology features.
Eighteen (3.5%) retinoblastoma patients developed primary phthisis bulbi. The median age of presentation was 1.5 years. The median duration of symptoms before presentation was 6 months. In total, 15 out of 18 (83%) cases had bilateral disease. Among these, 80% (12/15) had advanced intraocular disease in the fellow eye. Most common first symptom was white reflex. History of orbital inflammation was present in 12/18 cases. Computed tomographic scan of orbit showed intraocular mass with calcific densities in 16 eyes. In two cases, hyperdense mass was seen without any calcification. On histopathology, residual viable tumour cells with characteristics of poorly differentiated retinoblastoma were found in 67% (12/18) eyes. High-risk factors were present in six cases with microscopic residual disease in three cases.
This is the largest case series of retinoblastoma patients with primary phthisis bulbi. Phthisis bulbi in retinoblastoma may be associated with bilateral disease in most cases and advanced intraocular disease in the fellow eye in a significant number of cases. Regression is incomplete in majority of these cases; therefore, enucleation must definitely be done in all cases of retinoblastoma presenting with phthisis bulbi.
眼球萎缩是视网膜母细胞瘤一种相对罕见且非典型的临床表现。
在一家三级保健医院进行的回顾性研究。
18 例连续的原发性眼球萎缩的视网膜母细胞瘤患者。
对 2005 年 1 月至 2009 年 12 月在本中心治疗的所有原发性眼球萎缩的视网膜母细胞瘤患者的临床、影像学和组织病理学特征进行回顾性分析。
临床和组织病理学特征。
18 例（3.5%）视网膜母细胞瘤患者发生原发性眼球萎缩。发病时的中位年龄为 1.5 岁。在就诊前，中位症状持续时间为 6 个月。总共 18 例中有 15 例（83%）为双眼疾病。其中，80%（12/15）对侧眼存在晚期眼内疾病。最常见的首发症状是白色反光。18 例中有 12 例（67%）有眼眶炎症史。眼眶 CT 扫描显示 16 只眼有眼内肿块并伴有钙化密度。在 2 例中，可见高密度肿块但无任何钙化。组织病理学检查发现，67%（12/18）眼有残余的存活肿瘤细胞，具有低分化视网膜母细胞瘤的特征。6 例存在高危因素，其中 3 例有显微镜下残留疾病。
这是最大的一组原发性眼球萎缩的视网膜母细胞瘤患者病例系列。在大多数情况下，眼球萎缩与双侧疾病相关，在很大一部分病例中，对侧眼存在晚期眼内疾病。在这些病例中，大部分眼球萎缩不能完全消退，因此，对于所有出现眼球萎缩的视网膜母细胞瘤病例，必须行眼球摘除术。
