Objective:To observe the therapeutic effi cacy of chemotherapy combin ed with ophthalmic therapy on retinoblastoma (RB). Methods:The survival rate, eye ball remaining rate, and the control of the disease condition of 37 patients (56 eyes) with RB were retrospectively analyzed. The standard of the well contr ol of the disease included: (1) the ocular tumor shrank or even disappeared, and the tumor had creamlike changes or calcification and cicatrisation; (2) no oc u lar tumor recurrence in patients who had undergone enucleation; (3) no metastasi s found in the followup period. All of the patients had at least one eye with RB (ge;Ⅲb stage) underwent c hemotherapy. According to the response of the tumor to the chemotherapy, the pat ients generally underwent 6 times of systemic chemotherapy at regular intervals of 3-4 weeks. The medicines for chemotherapy included vincristine, cyclohosphamide , etoposide phosphate, and carboplatin. According to the self condition, the pati ents underwent chemotherapy combined with several ophthalmic therapies like phot ocoagulation, cryotherapy, transpupillary thermotherapy, 106Ru brachytherapy, en ucleation, etc. The observation duration lasted 2-59 months, with the average o f 35 months. Results:Thirty patients (83.3%) survived and were followed up, and 6 died (16.6%). One patient with bilateral tumor couldn't be followed up afte renucleation of both eyes. Among these 30 patients (45 eyes), eye ball remainin g rate at stage I-Ⅱ, Ⅲ-Ⅳ, and Ⅴ was 100% (10 eyes), 70% (10 eyes), and 14 .3% (21 eyes), respectively. In the followup duration, the disease in all of the 3 0 patients was controlled well. Conclusions:Chemotherapy combi ned with ophthalmic therapy is effective on RB.
ObjectiveTo observe the curative effect, survival rate, enucleation rate and pathological characteristics of retinoblastoma (RB) in children.MethodsRetrospective clinical study. From March 1999 to December 2018, a total of 313 patients (445 eyes) with RB diagnosed in Ophthalmology Department of Peking University People’s Hospital were enrolled in the study. Among them, 175 were male (55.9%), 138 were female (44.1%); 181 were monocular and 132 were binocular. The international standard of intraocular RB staging (IIRC) was 6, 13, 6, 52, 227 and 9 patients of A, B, C, D, E and extraocular stages respectively. Among the 313 patients, 245 patients were confirmed to the survivance, of which 22 cases (9.0%, 22/245) died. Among 445 eyes, 330 eyes definitely whether or not were enucleated; 184 eyes had definite IIRC stage, eye examination results, definite treatment plan and times before enucleation and definite pathological tumor node metastasis stage after operation. The basic information, demographic characteristics, clinical information, enucleation and treatment plan, pathological and immunohistochemical results were recorded. Binary logistic regression was used to analyze the risk factors of high risk pathological features (HRF) and prognosis in patients with RB.ResultsFrom 1999 to 2018, the survival rate of 245 patients was increased from 82.6% to 96.3% year by year; the enucleation rate of 330 eyes with final enucleation was reduced from 68.8% to 58.3% year by year. The rate of enucleation in stage D and stage E decreased from 83.3% and 100% before 2005 to 37.5% and 85.4% after 2014, respectively. Monocular disease (β=－1.551, P=0.005), stage D, stage E and extraocular stage in IIRC stage (P＜0.005) were the independent risk factors of RB enucleation, while the protective factors were Interventional chemotherapy of ophthalmic artery (IAC) (β=－0.877, P＜0.001). HRF was found in 51 eyes (27.7%). Age of onset (β=0.019, P=0.016) and glaucoma (β=0.816, P=0.050) were independent risk factors for HRF in RB pathology, while IAC treatment was the protective factor for enucleation (β=21.432, P＜0.001).ConclusionsAfter comprehensive treatment, the general trend of RB enucleation rate is gradually decreasing. IAC treatment can reduce the enucleation rate of stage D and E. The older age of onset and glaucoma stage are the independent risk factors of HRF, and IAC can reduce the risk factors of HRF.
ObjectiveTo observe and analyze the clinical characteristics of children who died of intraocular retinoblastoma (RB). MethodsA retrospective clinical study. Fourteen children (23 eyes) with intraocular RB who died after receiving treatment in Beijing Children's Hospital from 2009 to 2017 were included in the study. Among the children, there were 7 males (10 eyes) and 7 females (13 eyes); 5 had unilateral and 9 had bilateral tumor. Age were 17.2±15.5 months. All children underwent RetCam examination. RB was staged according to the international intraocular RB classify. Among the 23 eyes, 1 eye was in stage B, 2 eyes were in stage C, 12 eyes in stage D, and 8 eyes in stage E. Treatment methods included a systemic (vincristine, etoposide and carboplatin) chemotherapy (VEC chemotherapy), enucleation surgery, and vitrectomy. The basic conditions including age, time of diagnosis, pathological diagnosis, treatment and main causes of death were retrospectively analyzed. ResultsAmong the 14 cases, the first symptom was leukemia in 12 cases, red eye in 1 case, and squintin in 1 case. Systemic VEC chemotherapy was used for 1-6 courses of treatment; 5 cases were enucleated, 3 cases underwent histopathological examination; 3 cases were treated with vitrectomy. Among the 3 cases who underwent histopathological examination, the sclera and optic nerve, optic nerve and optic disc were invasted respectively. Seven patients died of tumor metastasis and/or intracranial lesions (50.0%, 7/14); the median survival time was 19 months. Four patients died of treatment (28.6%, 4/14), including 3 patients died of chemotherapy-related side effects, and 1 died of organ failure after enucleation surgery (7.1%); the median survival time was 3.5 months. Early abandonment of treatment died in 3 cases (21.4%, 3/14); the median survival time was 15 months. ConclusionIntracranial metastasis is the main cause of death in children with intraocular RB.
Retinoblastoma (RB) is the most serious eye disease that causes blindness, disability and death in infant. Loss of tumor suppressor gene RB1 leads to tumorigenesis of RB. With continuous innovation and development of new methods, the management of retinoblastoma has experienced a dramatic change from enucleation, external radiotherapy, intravenous chemotherapy, to intra-arterial chemotherapy combined with local treatment, enabling a more favorable outcome for survival, salvage and vision. However, the pathogenesis of RB is unclear in many aspects, the rate of eyeball removal in children with advanced RB remains high, and the innovation of new treatment are needed to be further promoted. Therefore, the key to improve the overall treatment level of RB is to pay attention to the study on pathogenesis, early diagnosis and treatment, as well as strengthen multi-center clinical trials and translational medicine research.
ObjectiveTo evaluate the pathological features of bilateral retinoblastoma (RB) and the relationship between different treatments and high-risk histopathologic features (HHF). MethodsRetrospective series of case studies. From 1999 to 2018, 73 patients with binocular RB diagnosed by pathological examination in Department of Ophthalmology, Peking University People's Hospital were included in the study. Among them, 50 patients were male (68.5%, 50/73), 23 patients were females (31.5%, 23/73); 11 patitents had a family history of RB. The mean age at the first diagnosis was 14.8±15.6 months. The average time between first diagnosis and first intervention was 3.97±4.74 months. According to the international classification standard of intraocular RB staging, among the 73 eyes, C, D and E stages were 2 (2.7%, 2/73), 15 (20.5%, 15/73), and 56 (76.7%, 56/73) eyes, respectively. Ocular images for each patient were obtained using a wide-angle contact fundus camera during examination under general anaesthesia. The treatment protocol (globe salvaging or enucleation) depended on the result of several clinical features. Globe salvaging treatment included chemotherapy combined with local therapy such as intra-arterial chemotherapy (IAC), intravitreal chemotherapeutics injection, cryotherapy, laser, transpupillary thermotherapy and radiotherapy. If globe salvaging failed, enucleation was offered and histopathologic analysis was conducted of the enucleated eye, the ophthalmic pathologist read and evaluated the presence of HHF. Independent samples t-test was performed to compare the continuous variables. The pathological features and the relationship between different treatments and HHF were analyzed. Group difference was calculated with chi-square. Results Among the 73 eyes, the first treatment was enucleation in 21 eyes (28.8%, 21/73); 52 eyes (71.2%, 52/73) were treated with eye protection. After enucleation, 9 cases (12.3%, 9/73) had recurrence and metastasis, and 7 cases (9.6%, 7/73) died. The intervention time of patients with recurrence and metastasis and those without recurrence and metastasis were 7.4±7.3 and 3.5±4.1 months respectively; the first intervention time of patients with recurrence and metastasis was significantly later than that of patients without recurrence and metastasis, but the difference was not statistically significant (t=－1.561, P=0.154). The pathological examination results showed that there were 26 eyes (35.6%, 26/73) with HHF, 4 (26.7%, 15/26) and 22 (39.3%, 22/56) eyes were in stage D and E, respectively. Those who received other treatments before enucleation had lower HHF percentages after enucleation than those who did not receive corresponding treatments, but the difference was not statistically significant (χ2=1.852, 0.074, 0.000, 1.007, 0.007, 2.729; P＞0.05). Among the 26 eyes, 5 (83.3%, 5/6) and 21 (31.3%, 21/67) eyes were treated with systemic chemotherapy combined with and without IAC, respectively, and there was a significant difference in the percentage of HHF (χ2=4.422, P=0.035). ConclusionsIAC eye-preserving therapy before enucleation has a significant effect on HHF.