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
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.