ABSTRACT
El Retinoblastoma es la neoplasia ocular más frecuente en pediatría. La Terapia radiante externa fue hasta hace una década el tratamiento conservador de elección. Luego se incluyó la quimio reducción; en un intento de evitar la radioterapia externa y sus complicaciones. En este estudio retrospectivo evaluamos los resultados del tratamiento conservador con terapia radiante externa o con quimio reducción en el servicio de oftalmología del Hospital Nacional de Pediatría Juan P. Garrahan, desde 1987 a 2009. De un total de 571 pacientes con diagnóstico de Retinoblastoma, 341 fueron unilaterales y 217 bilaterales. De estos últimos se analizaron 166 pacientes cuya edad media al diagnostico fue de 12 meses. Se trataron 332 ojos de 166 pacientes con Retinoblastoma bilateral, 157 ojos (47,3%) recibieron quimioreducción como tratamiento inicial, 115 ojos (34,6%) fueron enucleados al inicio, 45 ojos (13,6%) recibieron radioterapia externa como único tratamiento y 15 ojos (4,5%) recibieron tratamiento local solo (laser o crioterapia) como primera elección. Se analizaron los datos con el programa estadístico STATA 12.0 stataCorp Texas.USA. La agudeza visual final fue superior a 20/70 en el 51,5% de los pacientes e inferior en el 48,5%. Se encontró una relación significativa (p=0,005) entre el estadio al diagnóstico y la agudeza visual final; los pacientes con discapacidad visual se presentaron con estadios avanzados. Se evaluaron todas las orbitas enucleadas (157); de ellas el 74,5% recibieron radioterapia externa antes o después de la enucleación. El 24,8% de las orbitas irradiadas presentaron deformidad de la cavidad, con mala adaptación de prótesis y retracción orbitaria, el 70,1% presentaron cambios que permitían una adaptación de prótesis aceptable con alguna limitación de movilidad y solo 6 orbitas (5,12%) presentaban una muy buena cavidad para adaptación cosmética. Cuarenta de 157 orbitas enucleadas no recibieron radioterapia en ningún momento (25,5%), el 92,5% de ellas presentaron buena adaptación y solo el 7,5% tuvieron problemas de adaptación debido a complicaciones postoperatorias. El diagnóstico precoz, el tratamiento oportuno, y el uso de quimio reducción como terapia inicial en Retinoblastoma intraocular, permiten aumentar la tasa de preservación del globo ocular y reducen o eliminan la necesidad de recibir Terapia radiante externa, evitando sus secuelas (AU)
Retinoblastoma is the most common ocular neoplasia in childhood. External beam radiation therapy was the conservative treatment of choice until a decade ago. Subsequently, chemoreduction was added trying to avoid external beam radiation therapy and its complications. In this retrospective study we assess the results of conservative therapy with external beam radiation therapy or with chemoreduction at the Department of Ophthalmology at the Pediatric Hospital Juan P. Garrahan between 1987 and 2009. Of a total of 571 patients with a diagnosis of retinoblastoma, 341 had unilateral and 217 bilateral retinoblastoma. Of the latter patients, 166 patients were analyzed with a mean age at diagnosis of 12 months. Overall, 332 eyes of 166 patients with bilateral retinoblastoma were treated; at initial treatment 157 eyes (47.3%) underwent chemoreduction, 115 eyes (34.6%) were enucleated, 45 eyes (13.6%) underwent external beam radiation therapy as the only treatment, and 15 eyes (4.5%) only received local treatment (laser or cryotherapy) as a first choice. Data were analyzed using STATA 12.0 stataCorp Texas.USA. Final visual acuity was more than 20/70 in 51.5% and less in 48.5% of the patients. A significant relationship (p=0.005) between stage at diagnosis and final visual acuity was found; patients with visual impairment presented with advanced stages. All enucleated orbits were assessed (157); 74.5% underwent external beam therapy before or after enucleation. Of all irradiated orbits, 24.8% presented with cavity deformity, poor prosthesis fit, or contraction of the socket. Of all patients, 70.1% presented with changes that allowed acceptable fitting of the prosthesis with slight movement limitation and only 6 orbits (5.12%) had a good cavity for cosmetic appearance. Forty of 157 enucleated orbits did not receive radiation therapy at any moment (25.5%); 92.5% of them had a good fitting and in only 7.5% fitting problems due to postoperative complications were found. Early diagnosis, adequate treatment, and use of chemoreduction as initial therapy of intraocular retinoblastoma allow for an increased rate of preservation of the eye and reduce or eliminate the need for external beam therapy and its sequelae (AU)
Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Eye Enucleation , Radiotherapy/adverse effects , Retinal Neoplasms/classification , Retinal Neoplasms/drug therapy , Retinal Neoplasms/radiotherapy , Retinoblastoma/drug therapy , Retinoblastoma/radiotherapy , Follow-Up Studies , Retrospective StudiesABSTRACT
Retinoblastoma(RB) is the most common intraocular malignancy in children.The tumor can originate in one or both eyes.About 40％ of children with RB have inherited form of the disease.The treatment options available for RB include chemoreduction therapy,intra-arterial chemotherapy,radiotherapy,enucleation,genetherapy and focal therapies such as cryotherapy,laser photocoagulation,transpupillary thermotherapy.Currently,with the progress of early diagnosis and treatment,chemotherapy combined focal therapies play an important role in the treatment,which not only improve the survival rates of RB children,but also improve the children's life qualities with salvaging the eve and vision.
ABSTRACT
Retinoblastoma (RB) therapy has evolved over decades.In the 1970s, enucleation was important for improving life prognosis.In the 1980s, external beam radiotherapy (EBRT) was popular.In the 1990s, systemic intravenous chemotherapy (IVC) was introduced and currently remains prevalent worldwide for intraocular RB control as well as prevention of systemic metastasis.However, RB seeds in vitreous and subretinal space are still the major obstacles for successful treatments.Advanced ophthalmic imaging technology promotes thorough and detailed description and observation of RB seeds.In the 2000s, interests in periocular chemotherapy (POC), intra-arterial chemotherapy (IAC) and intravitreous chemotherapy (IVitC) have been explored to overcome the low drug concentration around the seeds and to reduce the systemic side effects.This leads us into a new era of target local chemotherapy of RB.How to make the treatment decision based on biological behavior of RB seeds is a major task for us now.
ABSTRACT
A 22-year-old female in her third trimester of pregnancy was referred to our department for sudden loss of vision with a painful blind eye. It was diagnosed as retinoblastoma clinically and radiologically. Histopathology and immunohistochemistry confirmed the diagnosis. This case is one of its kind because retinoblastoma occurring during pregnancy had not been reported in literature so far.
Subject(s)
Female , Humans , Pregnancy , Pregnancy Complications, Neoplastic/diagnosis , Retinal Neoplasms/diagnosis , Retinoblastoma/diagnosis , Young AdultABSTRACT
Intraocular (IO) retinoblastoma (RB) has traditionally been treated with enucleation (ENU) or external beam radiotherapy (EBRT). Recently, clinical trials are in progress to cure RB without ENU or EBRT in order to salvage the globe and to avoid unacceptable side effects of EBRT. We performed a pilot study to treat patients with advanced Reese-Ellsworth (RE) stage IO RB with initial chemotherapy (CRx) followed by local therapy (LT) and adjuvant CRx. Ten eyes (8 RE group V, 2 RE group IV) from 9 patients were enrolled from March 2001 to November 2001. All tumors responded to CRx. In 5 of 10 eyes, the RB was enough to be treated with LT after chemoreduction. One patient who underwent LT is waiting for ENU due to post-cryotherapy complication. For a median follow-up of 13 months (8-16 mo), 4 eyes that received LT and adjuvant CRx were relapse-free. A patient with bilateral RB who failed to be a candidate for LT was rescued with high-dose CRx and hematopoietic stem cell transplantation. Consequently, by treating patients according to our strategy, we were able to salvage 6 out of 10 eyes without ENU or EBRT. These results suggest that chemoreduction followed by LT and adjuvant CRx might offer the opportunity to salvage the globe and vision even in patients with advanced stage IO RB.