ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
Nowadays,one of the most challenging aspects of retinoblastoma (RB) therapy is how to control the resistant or recurrent viable vitreous seeds,for which intravenous chemotherapy appears to be ineffective.Recently,intravitreal chemotherapy offers another option to control advanced stage and vitreous seeds of RB,and may be a promising new approach to RB therapy.However,intravitreal injection for RB patients raises considerable controversy due to concerns of possible extraocular extension along the injection route,and should not replace the primary standard of care for bilateral RB or group E eyes of RB.Close follow-up and further studies are needed to determine appropriate indications,to determine the effective drugs and concentrations,to optimize RB therapy protocols and to investigate the relationship between long-term efficacy and toxicities.
ABSTRACT
Objective To observe the effect of systemic chemotherapy on conditions of tumor infiltrating,metastasis and disease-specific survival (DSS) for advanced retinoblastoma (RB).Methods Forty-one patients with advanced RB who received enucleation were enrolled in this study.There were 26 males and 15 females,age at diagnosis was ranged from 2 to 72 months,with a mean of 23.08 months.There were 16 bilateral patients and 25 unilateral patients;13 group D eyes and 28 group E eyes.16 patients received enucleation as the primary treatment (operation group),25 eyes received chemotherapy before enucleation (chemotherapy group).There was no significant statistical difference between two groups for the gender,unilateral and bilateral,international staging or diagnostic age (P＞0.05).The histopathology report was performed to assess the risk of postoperative tumor-node-metastasis staging (pTNM) in each patient,and the extent of tumor invasion in the optic nerve,choroid and anterior chamber was divided into 3 levels of low risk,medium risk and high risk.Five deaths were all in the group E with chemotherapy before enucleation.Using R software survival analysis software package survfit function,the application of Kaplan-Meier estimation method,DSS of RB children was calculated from the time of diagnosis,up to the date of the death of patient.DSS differences between chemotherapy,operation group and eye removal time (more than 3 months,less than 3 months) in group E RB children were analyzed.Results The proportion of high risk pTNM stage in chemotherapy group was significantly lower than the operation group.But there was no significant difference between the two groups in the overall risk classification (x2 =3.130,P=0.077).For group D eyes,the overall risk classification in chemotherapy group was significantly lower than the operation group (x2 =5.870,P=0.015).There was no significant difference between the two groups in the overall risk of group E eyes (x2 =0.020,P=0.889).The DSS in chemotherapy group and operation group were 0.71 and 1.00,respectively;the difference was significant (x2 =3.700,P=0.05).The DSS in children whose enucleation delayed for more than 3 months and children whose enucleation performed within 3 months were 0.64 and 1.00,respectively;the difference was significant (x2 =4.800,P=0.028).Conclusion Systemic chemotherapy did not reduce the risk of tumor invasion and metastasis in patients with advanced RB.Instead,it will reduce the DSS in group E eyes of RB.
ABSTRACT
A 4 year-old girl with bilateral, non-familial retinoblastoma (RB) was referred to our care after primary enucleation OS and active tumor OD refractory to multiple therapies (intravenous chemotherapy, laser/cryotherapy, and I-125 plaque radiotherapy). Vitreous seeding OD, initially controlled by several sessions of Ophthalmic Artery Infusion Chemotherapy (OAIC) and periocular chemotherapy, recurred shortly thereafter. The patient underwent intravitreal (IVit) Melphalan injections achieving tumor control despite the concurrent development of keratopathy, pupillary synechiae, cataract, and necrosis of the inferior fornix and the adjacent orbital fat, all secondary to the treatments administered. Repeated amniotic membrane implants and tarsorrhaphy were performed to alleviate the symptoms. Despite being tumor free for 6 months, a poor fundus view and treatment-related complications prompted us to consider enucleation, but parents declined. Following recent negative magnetic resonance imaging (MRI), her cataract was removed. She was then found to have tumor recurrence. Her eye was enucleated 12 months ago and she recovered well from the surgery. As ocular oncology embarks in eye-preserving treatments for retinoblastoma, it is important to address the cumulative effects and associated impact of such treatments and the possibility of failure.
Uma menina de 4 anos com retinoblastoma (RB) bilateral, não-familiar foi encaminhada após enucleação OE e tumor ativo OD refratário a múltiplas terapias (quimioterapia endovenosa, laser/crioterapia e braquiterapia com I-125). Semeadura vitrea OD, inicialmente controlada por inúmeras sessões de Quimioterapia Intra-Arterial Oftálmica (QIAO) e quimioterapia periocular, recorreu em seguida. Paciente recebeu injeções intravítreas de Melphalan obtendo controle tumoral apesar do desenvolvimento concomitante de ceratopatia, sinéquias pupilares, catarata, necrose do fórnice inferior e gordura periorbitária adjacente, todos secundários aos tratamentos usados. Implantes repetidos de membrana amniótica e tarsorrafias foram realizadas para melhora sintomatológica. Apesar de estar livre de tumor por 6 meses, a baixa visibilidade do fundo e complicações terapêuticas nos levaram a considerar enucleação que foi descartada pelos pais. Após recente ressonância magnética nuclear (RMN) negativa, a catarata foi removida. Foi então detectada recorrência tumoral. O olho foi enucleado há 12 meses e ela se recuperou bem da cirurgia. Enquanto a oncologia ocular embarca em tratamentos para preservar em retinoblastoma, é importante considerar os efeitos cumulativos e impacto associado desses tratamentos, e a possibilidade de fracasso.
Subject(s)
Child, Preschool , Female , Humans , Neoplasm Recurrence, Local/therapy , Retinal Neoplasms/therapy , Retinoblastoma/therapy , Combined Modality Therapy/adverse effects , Combined Modality Therapy/methods , Eye EnucleationABSTRACT
Retinoblastoma (RB) is the most common intraocular malignant tumor in children. With advanced clinical technologies there are more and more methods available to treat retinoblastoma, and make it is possible to delivery individualized protocol combined traditional treatments with modern regimen to patients now. In order to improve the survival rate and the life quality of RB patients in China, it is very important to make a suitable system of standardized therapy based on results from developed countries and health policies of our own country.