ObjectiveTo evaluate the efficacy of intra-arterial chemotherapy (IAC) for advanced retinoblastoma (RB) after failure of intravenous chemotherapy (IVC). MethodsFifteen eyes of 13 patients with advanced RB were treated with IAC (1-5 cycles) after failure of IVC (2-8 cycles). The patients included 10 boys and 3 girls, with the mean age of (15.67±8.16) months. Six patients had bilateral RB and 7 patients had unilateral RB. There were 14 eyes (93.33%) in stage D, 1 eye (6.67%) in stage E according to the International Classification of intraocular retinoblastoma. The main reasons for failure of IVC were recurrent primary tumor in 3 eyes (20.00%), subretinal seeds recurrence in 9 eyes (60.00%), viable vitreous seeds in 2 eyes (13.33%) and poor response of primary tumor in 1 eye (6.67%). The mean interval between IVC completion and IAC start was 3 months. The mean follow-up was 19 months (ranged from 3 to 52 months). ResultsAfter IVC and secondary IAC, the retinoblastoma and seeds were regressed in 12 eyes (80.00%). Three eyes required enucleation for severe vitreous seeds, subretinal seeds recurrence and primary tumor recurrence. There was no evidence of metastasis in any case. ConclusionIAC can achieve high global salvage rate (80.00%) for patients with advanced retinoblastoma after failure of IVC.
Intra-arterial chemotherapy (IAC) is an interventional treatment for retinoblastoma (RB) which infuses the chemotherapeutic agents through ophthalmic artery using microcatheters to control the tumor. Compared with systemic chemotherapy, IAC could significantly increase the globe salvage of advanced and recurrent RB without serious adverse event. Due to the absence of systemic absorption after IAC, no longer effectively kill tumor cell, which have potential danger to leads to inadequate elimination extraocular tumor cells. The most common systemic complications following IAC is myelosuppression; local ocular toxicities include eyelid edema, delacrimation, blepharoptosis, vitreous hemorrhage, retinal artery obstruction. During the last 10 years of clinical application, IAC become one of first-line treatment for intraocular RB. However, at present, there is still a lack of randomized controlled multicenter studies and long-term follow up of IAC.
Objective To analyze the efficacy and safety of Intra-arterial chemotherapy (IAC) as secondly treatment in children with retinoblastoma (RB). Methods 42 eyes of 34 consecutive RB patients were enrolled in the study after intravenous chemotherapy (IVC), including 26 males and 8 females. The average age is 14.1 months. 21 cases were bilateral and 7 cases were unilateral. A total of 42 eyes of 34 patients were classified according to the International Intraocular Retinoblastoma Classification（IIRC）as group B(n=1, 2.4%), group C (n=3, 7.1%), group D (n=32, 76.2%), or group E (n=6, 14.3%). Tumor recurrence and tumor enlargement after IVC were 4 and 10 eyes respectively, accounting for 9.0% and 24.0% respectively. Sequential treatment after IVC followed by IAC were 28 eyes, accounting for 67.0%. All treatment eyes received IAC combined with laser, cryotherapy and other eye local treatment. The IAC regimen adopted the combination and alternation administration mode, by the combination of melphalan and carboplatin or the combination of melphalan and topotecan. According to the tumor changes after IAC decide whether IAC again. If tumors increased, vitreous or subretinal implants increased will be termination of IAC and enucleation. The mean follow-up time was (21.4±3.7) months after the last IAC treatment and (6.2±2.9) months after enucleation. Ocular preservation rate and complication were evaluated. Results The average IAC procedures performed on 42 eyes were (4.0±0.9). An overall ocular preservation rate of 76.2% was observed during follow-up periods due to calcification or inactivation of tumors (32 eyes), including group B (n=1, 100%), group C (n=1, 33.3%), group D (n=27, 84.4%), group E (n=3, 50%). 10 eyes were enucleated. Among them, 2 eyes of the tumor did not shrink after IAC, tumor recurrence (n=3), vitreous hemorrhage (n=3), enophthalmos (n=1), vitreous disseminated (n=1). 34 cases of children, transient eyelid oedema were 18 cases, vitreous hemorrhage and bone marrow suppression (Ⅰ-Ⅳ) were 1, 22 casese respectively. Conclusions IAC as secondly treatment is safe and effective for RB patients, however, there is still tumor recurrence. No serious ocular local and systemic complications were observed.
Thanks to the treatment of retinoblastoma (RB) having improved significantly in recent years, there is an increasing trend to use conservative treatment modalities that aim to preserve the globe as well as vision with minimum mortality. RB therapy is a long-term systemic treatment in clinical practice. Although there are many treatment options for RB therapy, such as cryotherapy, photocoagulation, systemic chemotherapy, enucleation and ophthalmic chemotherapy, it is recommended to consider in accordance with the following key points in gaining a reasonable treatment strategies: to make sure that RB is an intraocular period; to determine whether the intraocular RB to be treated with eye preservation or enucleation; what is the case of eye preservation therapy combined with chemotherapy and how to arrange the follow-up of RB patients. It's more complicated to choice the therapeutic measures for RB in clinical practice. So, the patient's condition, economic capability and medical condition should be evaluated comprehensively. The principle of RB treatment should be followed, which is protecting eyeball and visual function without life damage.
ObjectiveTo observe the clinical characteristics of rhegmatogenous retinal detachment (RRD) secondary to conservative therapy in retinoblastoma (RB) patients.MethodsA retrospective study. From July 2013 to May 2017, 20 RRD patients (20 eyes) of 456 RB patients (573 eyes) treated in Xinhua Hospital of Shanghai Jiao Tong University School of Medicine were included in the study. Eleven patients (11 eyes) were boy and 9 patients (9 eyes) were girls. Thirteen patients demonstrated bilateral RB and 7 patients had unilateral RB. Average age when diagnosed with RB was 25 months. International Classification of Retinoblastoma groups were C in 1 eye, D in 17 eyes, and E in 2 eyes. These patients received intra-arterial chemotherapy (17 eyes), intravenous chemotherapy (11 eyes), intravitreal chemotherapy (8 eyes), laser (14 eyes) and/or cryotherapy (5 eyes). Twelve patients (12 eyes) received vitreoretinal surgery including vitrectomy (6 eyes) and scleral buckling (7 eyes). The mean follow-up was 39 months. Fundus examination was performed under general anesthesia during comprehensive treatment and follow-up. The time interval of fundus examination varied from 1 to 6 months depending on the stability of the tumor.ResultsRRD was noted in 20 eyes (3.5%) with RB. Retinal hole was found in 15 eyes (75%). The cause of RRD was atrophic hole in calcified tumor (6 eyes, 30%), cryotherapy-related hole (5 eyes, 25%) and laser-related hole (9 eyes, 45%). Multiple atrophic hole in calcified tumor was noted in 3 eyes. Size of hole smaller than 2 DD was noted in 8 eyes (53%), and larger than 2 DD was noted in 7 eyes (47%). Holes were in posterior (3 eyes), equator (2 eyes) and periphery (10 eyes). Severe proliferated was noted in 1 eye. No tear was found. No bulbar retinal detachment and choroidal detachment was noted. Among 12 eyes who underwent vitreoretinal surgery, reattachment was achieved in 9 eyes (75%). No metastasis was noted.ConclusionsCalcified regression of tumor, cryotherapy and laser were main reasons of RRD. Most of the holes are small in diameter and located in the periphery.
ObjectiveTo observe the clinical characteristics of retinoblastoma (RB) in Southwest China.MethodsA retrospective clinical study. From January 2010 to December 2017, 66 RB patients diagnosed in Ophthalmology Department of West China Hospital of Sichuan University were included in the study. All the patients underwent ocular B-ultrasound, orbital CT or MRI examination. Ten patients underwent RetCam examination at the same time. Twenty-nine patients were diagnosed by histopathological examination, and 37 patients were diagnosed by clinical symptoms and imaging examination. According to whether the tumor invaded the orbit and optic nerve, it could be divided into extraocular stage and intraocular stage. Intraocular tumors were divided into A-E stages according to the international intraocular RB classification. Treatments were performed according to different stages. The general information, age at diagnosis, course of diseases (the time between onset symptoms and diagnosis), causes of visiting a doctor, classification, treatment methods and eyeball preservation rate were retrospectively analyzed.ResultsPatients all came from Southwest China (56 patients from Sichuan Province, 2 patients from Yunnan Province, 2 patients from Guizhou Province, and 6 patients from Tibet). The permanent residence were identified in 43 patients, including 27 patients (62.8%) from rural areas. There were 38 males (57.6%); 50 unilateral tumors (75.8%) and 16 bilateral tumors (24.2%); 51 first-visiting patients (77.3%) and 15 re-visiting patients (22.7%). The average diagnostic age of first-visiting patients was 20.9±14.4 months, with 23.2±14.7 and 11.2±7.6 months for unilateral and bilateral tumors, respectively. There were 41 patients had definite course and causes, of whom the average course was 90.6±115.2 days. The most common cause was leucocoria in 32 patients (62.7%), followed by redness and swelling in 4 patients (9.8%), and other causes in 5 patients (12.2%). Among the 15 re-visiting patients, the average diagnostic age was 63.6±46.8 months, the average course was 32.8±45.5 months. Recurrence was occurred in 5 patients (33.3%), leucocoria in 4 patients (26.7%), postoperative complication in 3 patients (20.0%), protrusion in 2 patients (13.3%) and redness in 1 (6.7%) patient, respectively. Fifty out of 82 eyes were admitted to hospital, including 37 eyes of first-visiting patients and 13 eyes of re-visiting patients. Among 37 first-visiting eyes, there were 5 eyes (13.5%) in stage A-C, 26 eyes (70.3%) in stage D-E, 6 eyes (16.2%) in extraocular stage. Five eyes in stage A-C were treated with laser photocoagulation and (or) cryotherapy combined with systemic chemotherapy. Four eyes in stage D were treated with intraocular arterial chemotherapy. Nineteen eyes (51.3%) were performed with enucleation, 2 eyes (5.4%) with evisceration and 7 eyes (18.9%) abandoned treatment. Among 13 re-visiting eyes, 6 eyes (46.2%, with 5 eyes of recurrence) had been enucleated before, 4 eyes (30.8%) were in extraocular stage and 3 eyes (23.1%) in stage D-E. Five eyes (38.5%) were treated with evisceration, 4 eyes (30.8%) with enucleation, 1 eye with oculoplastic surgery and 3 eyes (23.1%) abandoned treatment. The rate of eye preservation was 18.0%, 29.0% for intraocular stage and 0% for extraocular stage, respectively.ConclusionRB patients in Southwest China have a longer course between onset symptoms and diagnosis, more advanced classification and lower rate of eye preservation.
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 compare the clinical efficacy and complications of intra-arterial chemotherapy (IAC) and intravenous chemotherapy (IVC) for unilateral advanced retinoblastoma (RB). MethodsA retrospective clinical study. From January 2020 to January 2021, 40 patients (40 eyes) unilateral group cT2 RB patients diagnosed at Baoding Children’s Hospital and Beijing Children’s Hospital were recruited in this study. There were 22 males (22 eyes) and 18 females (18 eyes). All were monocular. All the patients were assigned to two groups according to different treatment modalities they received: IVC group and IAC group. There were 26 eyes and 14 eyes, respectively. When the tumor invades the optic nerve, choroid, sclera, anterior chamber and iris, enucleation was performed. The globe salvage rate, tumor extraocular metastasis rate, solid tumor control rate, treatment-related complications and pathological high-risk factors after enucleation were observed. The globe salvage rate and solid tumor control rate were compared between the groups by chi square test. ResultsThe globe salvage rate of IAC group and IVC group were 88.5% (23/26) and 50.0% (7/14), respectively. Solid tumor control of IAC group and IVC group were 84.6% (22/26) and 42.9% (6/14), respectively. There were statistically significant differences in globe salvage rate and solid tumor control between the two groups (χ2=7.18, 7.56; P＜0.05). Compared with IVC group, IAC group had less systemic complications, mild ocular and periocular side effects. Among 26 cases in IAC group and 14 cases in IVC group, 3 and 7 cases underwent enucleation respectively. The results of pathological examination showed that there were 2 cases and 3 cases with pathological high-risk factors in the two groups, respectively. During the follow-up period, 2 cases in IAC group had extraocular metastasis, there was no extraocular metastasis in IVC group. ConclusionCompared with IVC, IAC has the advantages of high tumor control rate, high globe salvage rate, less and mild complications, however, there is still tumor recurrence.
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.