Vasculopatía coroidea oclusiva tras quimioterapia intraarterial: hallazgos en la RMN / Choroidal occlusive vasculopathy after intraarterial chemotherapy: MRI findings

Objetivo: Evaluar los hallazgos en las imágenes de resonancia magnética nuclear (RMN) en pacientes con vasculopatía coroidea oclusiva (VCO) tras quimioterapia intraarterial (QIA) por retinoblastoma. Métodos: Se realizó un estudio retrospectivo de 37 ojos de 34 pacientes que recibieron QIA entre 2016 y 2021 como tratamiento de primera o segunda línea del retinoblastoma intraocular. De estos pacientes, 22 recibieron quimioterapia sistémica y el resto QIA como primera línea, con melfalán (3-4mg), carboplatino (40mg) y topotecan (20mg). Los pacientes fueron examinados cada mes para observar la regresión tumoral y posibles complicaciones de los tratamientos. A los pacientes que presentaron VCO se les realizaron estudios con RMN para evaluar el grosor coroideo y la longitud del globo ocular. Resultados: Se observó VCO en cinco de los 37 ojos (13,51%), todos ellos con una coroidopatía sectorial completa con afectación foveal (grado 2). En cuatro de los cinco pacientes el grosor coroideo se vio disminuido, mientras que en tres casos el tamaño del globo afectado era claramente inferior. El control tumoral fue posible en todos los casos. Conclusiones:En esta muestra, la VCO se asocia con adelgazamiento coroideo y diminución del tamaño ocular en la RMN. Puede ser necesaria una nueva clasificación para correlacionar mejor la severidad de la coroidopatía que afecta a la fóvea. Los resultados iniciales son favorables respecto al uso de la QIA; aunque es necesaria la realización de estudios a largo plazo y una documentación exhaustiva para valorar tanto el papel de la QIA, como los efectos derivados de ella. (AU)

Purpose: To evaluate magnetic resonance imaging (MRI) findings in patients suffering choroidal occlusive vasculopathy (COV) after intra-arterial chemotherapy (IAC) for retinoblastoma. Methods: A retrospective study of 37 eyes of 34 patients receiving IAQ between 2016 to 2021 as primary or secondary treatment for retinoblastoma was conducted. Twenty-two patients received systemic chemotherapy with carboplatin, vincristine and etoposide. The rest received IAC as primary treatment. The drugs administered were melphalan (3-4mg), carboplatin (40mg) plus topotecan (20mg). The patients were examined under general anaesthesia every month to observe tumor regression and possible complications of the treatment. For the patients with COV an MRI was obtained to analyse the choroidal thickness and axial ocular length. Results: A COV was observed in 5 of the 37 eyes receiving IAC (13,51%), all of them with a complete sectorial choroidopathy not sparing the fovea (grade 2). In 4 of the 5 patients the choroidal thickness was decreased and in three cases the size of the eye which presented COV was clearly smaller than the contralateral eye. Tumor control was archived in all 5 patients. Conclusion: In our cases COV was associated with reduction of thinning of choroid and eye length in the MRI. A new classification maybe needed to correlate better with the severity of the complication affecting the fovea. Although early results generally are favorable to the use of IAC, longer follow up and scrupulous documentation of side effects will be necessary to know the true role of IAC for retinoblastoma. (AU)

Choroidal occlusive vasculopathy after intraarterial chemotherapy: MRI findings.

PURPOSE: To evaluate magnetic resonance imaging (MRI) findings in patients suffering choroidal occlusive vasculopathy (COV) after intra-arterial chemotherapy (IAC) for retinoblastoma. METHODS: A retrospective study of 37 eyes of 34 patients receiving IAQ between 2016 to 2021 as primary or secondary treatment for retinoblastoma was conducted. Twenty-two patients received systemic chemotherapy with carboplatin, vincristine and etoposide. The rest received IAC as primary treatment. The drugs administered were melphalan (3-4mg), carboplatin (40mg) plus topotecan (20mg). The patients were examined under general anaesthesia every month to observe tumor regression and possible complications of the treatment. For the patients with COV an MRI was obtained to analyse the choroidal thickness and axial ocular length. RESULTS: A COV was observed in 5 of the 37 eyes receiving IAC (13,51%), all of them with a complete sectorial choroidopathy not sparing the fovea (grade 2). In 4 of the 5 patients the choroidal thickness was decreased and in three cases the size of the eye which presented COV was clearly smaller than the contralateral eye. Tumor control was archived in all 5 patients. CONCLUSION: In our cases COV was associated with reduction of thinning of choroid and eye length in the MRI. A new classification maybe needed to correlate better with the severity of the complication affecting the fovea. Although early results generally are favorable to the use of IAC, longer follow up and scrupulous documentation of side effects will be necessary to know the true role of IAC for retinoblastoma.

Intra-arterial chemotherapy for retinoblastoma treatment in Chile: Experience and results 2013-2020.

OBJECTIVE: To establish the success rate of salvage intra-arterial chemotherapy (IAC), defined as the percentage of eyes that achieved tumoral remission and avoided enucleation. The second objective was the clinical characterization, catheterization results, and associated local and systemic complications. METHODS: Retrospective, interventional case series of 29 patients (35 eyes) with persistent or recurrent retinoblastoma. RESULTS: A total of 73 salvage IAC procedures with topotecan and melphalan were carried out. Success rate was 77% at a mean follow-up of 41.4 months. All patients with only one remaining eye avoided enucleation (10 cases). Catheterization was successful in 98.6% of cases. The types of catheterizations were as follows: 71.2% supraselective ophthalmic artery, 12.3% occlusion pump assisted supraselective ophthalmic artery, 16.4% selective external carotid with retrograde flow. 14% of patients suffered local adverse effects: 1 (2.8%) transitory ptosis, 1 (2.8%) transitory oculomotor nerve palsy, 2 (5.7%) aseptic cellulitis and 1 (2.8%) periorbitary pigmentation. 4.1% (3 cases) suffered neutropenia due to medullar chemosuppression. There were no cases of severe anemia or thrombocytopenia. There were no cerebral ischemic events or mortality associated to the procedure. CONCLUSION: IAC with melphalan and topotecan is a safe and effective treatment option for persistent or recurrent retinoblastoma, able to reduce enucleation rates.

Quimioterapia intraarterial para el tratamiento del retinoblastoma en Chile: experiencia y resultados, período 2013-2020 / Intra-arterial chemotherapy for retinoblastoma treatment in Chile: experience and results 2013-2020

Objetivo Establecer la tasa de éxito de la quimioterapia intraarterial (QIA) en modalidad de rescate expresada en el porcentaje de ojos que lograron remisión tumoral y evitaron la enucleación. El segundo objetivo fue analizar la caracterización clínica, resultados del cateterismo, complicaciones locales y sistémicas asociadas. Métodos Estudio retrospectivo de serie de casos intervencional de 29 pacientes (35 ojos) con retinoblastoma intraocular persistente o recidivado. Resultados Se realizaron un total de 73 procedimientos de QIA con topotecán y melfalán en modalidad de rescate. La tasa de éxito de la QIA fue del 77% en un seguimiento promedio de 41,4 meses. Todos los casos con ojo único evitaron la enucleación de su ojo residual (10 casos). El cateterismo fue exitoso en un 98,6%. Los tipos de cateterización logrados fueron los siguientes: supraselectiva por arteria oftálmica (71,2%), supraselectiva por arteria oftálmica asistida con balón de oclusión en carótida externa (12,3%), selectiva por rama de carótida externa con flujo retrógrado (16,4%). Se reportaron efectos adversos locales en el 14% de los pacientes: una (2,8%) ptosis palpebral transitoria, una (2,8%) parálisis del sexto par transitoria, 2 (5,7%) celulitis aséptica y una (2,8%) pigmentación de región periorbitaria. La frecuencia de neutropenia por quimiosupresión medular fue del 4,1% (3 casos). No hubo casos de anemia ni trombocitopenia severas. No hubo eventos isquémicos cerebrales ni mortalidad asociada al procedimiento. Conclusión La QIA con melfalán y topotecán es una alternativa segura y efectiva para el tratamiento del retinoblastoma persistente o recidivado, permitiendo reducir las tasas de enucleación (AU)

Objective To establish the success rate of salvage intra-arterial chemotherapy (IAC), defined as the percentage of eyes that achieved tumoral remission and avoided enucleation. The second objective was the clinical characterization, catheterization results, and associated local and systemic complications. Methods Retrospective, interventional case series of 29 patients (35 eyes) with persistent or recurrent retinoblastoma. Results A total of 73 salvage IAC procedures with topotecan and melphalan were carried out. Success rate was 77% at a mean follow-up of 41.4 months. All patients with only one remaining eye avoided enucleation (10 cases). Catheterization was successful in 98.6% of cases. The types of catheterizations were as follows: 71.2% supraselective ophthalmic artery, 12.3% occlusion pump assisted supraselective ophthalmic artery, 16.4% selective external carotid with retrograde flow. 14% of patients suffered local adverse effects: 1 (2.8%) transitory ptosis, 1 (2.8%) transitory oculomotor nerve palsy, 2 (5.7%) aseptic cellulitis and 1 (2.8%) periorbitary pigmentation. 4.1% (3 cases) suffered neutropenia due to medullar chemosuppression. There were no cases of severe anemia or thrombocytopenia. There were no cerebral ischemic events or mortality associated to the procedure. Conclusion IAC with melphalan and topotecan is a safe and effective treatment option for persistent or recurrent retinoblastoma, able to reduce enucleation rates (AU)

[Multidisciplinary management of retinoblastoma: Experience in 37 eyes]. / Manejo multidisciplinario en retinoblastoma: experiencia en 37 ojos.

OBJECTIVE: To report the results of the multidisciplinary management of patients with retinoblastoma, including survival, enucleation rate, and systemic chemoreduction success. METHODS: A retrospective study was conducted on 27 patients (37 eyes) diagnosed with retinoblastoma, and treated by a multidisciplinary team in San Juan de Dios Hospital. Demographic information, clinical characterization, survival, local and systemic treatments were included in the analysis. Patients treated with intra-arterial chemotherapy (IAC) were also reviewed. RESULTS: The study included14 male patients (52%). The median of age at presentation was 8 months (0.16-90). The median follow-up time was 33 ± 21 months. The diagnosis was made in 10 (37%) cases after 15 months old, with a median of 35 months (24-90). 17 (63%) patients had unilateral retinoblastoma, and 10 (37%) bilateral retinoblastoma. Leukocoria, isolated or associated with other signs, was the most frequent reason for referral (63%). Global enucleation rate was 57% (n=21), being the primary treatment in 15 (55%) patients. Enucleation rate in unilateral retinoblastoma was 76.5%, and for bilateral retinoblastoma, it was 60% for one eye and 10% for both. Systemic chemotherapy was prescribed in 17 (63%) patients, with a mean number of cycles of 5.3 ± 2.1. The overall success of chemoreduction and focal therapy in order to avoid external radiotherapy and/or enucleation was 68%. Three patients were treated with IAC as a salvage therapy, controlling the tumor in 2 patients at 6 months of follow-up. These are the first cases reported in Chile. Survival rate was 100%. CONCLUSION: Multidisciplinary management of retinoblastoma led to a survival rate and morbidity comparable with international reports.

Supervivencia libre de enucleación de pacientes con diagnóstico de retinoblastoma tratados con quimioterapia intraarterial en el Hospital Universitario de San Vicente Fundación / Enucleation free survival of patients with retinoblastoma diagnosis treated with intraarterial chemotherapy at University-Based San Vicente Fundacion Hospital

Objetivo: describir la supervivencia libre de enucleación, respuesta al tratamiento y complicaciones de los pacientes sometidos a tratamiento con quimioterapia intraarterial (QIA) para el control del retinoblastoma en el Hospital Universitario de San Vicente Fundación (HUSVF). Tipo de estudio: estudio de cohortes. Materiales y Métodos: se incluyeron todos los pacientes con diagnóstico de retinoblastoma tratados en el husvf con qia supraselectiva de la arteria oftálmica entre agosto del 2011 y agosto de 2013. Resultados: se realizó tratamiento a 21 pacientes, 24 ojos, en un periodo de 23.5 meses. La edad media al momento de inicio de la QIA fué 19 meses (rango 5-60 meses) con un seguimiento promedio de 9.3 meses (rango 3 semanas a 23.5 meses). Se realizaron 78 cateterizaciones, siendo 9 de ellas bilaterales. El salvamento ocular se alcanzó en el 100% de los Grupo A (n1), 100% B (n=2), 100% C (n=5) y 87.5% D (n=16), según la Clasificación Internacional para Retinoblastoma (CIRB). Sólo se reportaron dos eventos adversos importantes, una hemiparesia transitoria y una reacción alérgica severa. Ninguno de los pacientes ha fallecido, ha tenido compromiso metastásico o necesidad de radioterapia externa. Sólo se realizaron dos enucleaciones. La media de supervivencia de los 24 globos oculares tratados es de 10.4 meses ± 1.6. La media de supervivencia de los 21 pacientes tratados es de 10.4 meses ±1.7. Conclusiones: los resultados muestran una respuesta satisfactoria, constituyéndose la QIA como procedimiento seguro y con baja tasa de complicaciones en el tratamiento del retinoblastoma.

Purpose: to describe globe salvage, treatment response and complications of patients following intra-arterial chemotherapy (IAC) for retinoblastoma at Hospital Universitario de San Vicente Fundacion (HUSVF). Type of study: Cohort Study. Materials and methods: We included all patients diagnosed with retinoblastoma and treated with IAC between from August 2011 to August 2013. Results: A total of 21 patients, with a total of 24 eyes, were treated with IAC over a period of 23.5 months. The mean age of patients at time of treatment was 19 months (range 5-60 months) with a mean follow-up time of 9.3 months (range 3 weeks to 23.5 months). 78 catheterizations were performed, nine of them being bilateral. Ocular salvage rate was achieved in 100% of the Group A (n1), 100% in group B (n = 2), 100% in group C (n = 5) and 87.5% in group D (n = 16), according to the International Classification for Retinoblastoma. Only two major adverse events were reported, a transient hemiparesis and a severe allergic reaction. No deaths, metastatic involvement or need for external beam radiotherapy were reported. Two eyes, from group D, required enucleation. The mean survival rate of 24 eyes treated was 10.4 months ± 1.6. The mean survival rate of the 21 patients treated was 10.4 ± 1.7 months. Conclusions: The results show a satisfactory response, becoming the IAC a safe procedure with a low complication rate in retinoblastoma treatment.