ABSTRACT Iridociliary ring melanoma is an uncommon type of uveal melanoma. Clinical manifestation varies from asymptomatic cases to masquerade syndromes mimicking refractory glaucoma. Treatment options include radiotherapy and enucleation. Management of metastatic uveal melanoma remains discouraging. Novel therapies using immune checkpoint inhibitors are currently under study. We present a case of a 54-year-old Hispanic woman with progressive vision loss due to metastatic ring melanoma with anterior chamber seeding treated with pembrolizumab.

RESUMO O melanoma iridociliar em anel é um tipo incomum de melanoma uveal. As manifestações clínicas variam desde casos assintomáticos até síndromes mascaradas que mimetizam um glaucoma refratário. As opções de tratamento incluem radioterapia e enucleação. O manejo do melanoma uveal metastático continua desanimador. Novas terapias usando inibidores de checkpoint imunológico estão atualmente em estudo. Apresentamos o caso de uma mulher hispânica de 54 anos com perda progressiva da visão por um melanoma metastático em anel, com semeadura de câmara anterior, tratada com pembrolizumabe.

Resumen Objetivo: Reportar los resultados iniciales de la cirugía estereotáctica con CyberKnife® (CK®) para etapas avanzadas de melanoma de cuerpo ciliar y coroides (MCC-C) en pacientes mexicanos. Método: Revisión retrospectiva de expedientes clínicos. Se analizaron los resultados de los pacientes a quienes se trató con CK® (Accuray Inc, Sunnyvale, CA, Estados Unidos). Resultados: Cuatro pacientes con MCC-C fueron tratados bajo este protocolo. La media de edad fue de 53.2 ± 5.3 años (rango: 47-60). La mediana de seguimiento fue de 9.5 ± 3.1 meses (rango: 5-12). El diámetro promedio del tumor fue de 13.49 mm, el grosor promedio fue de 11.74 mm y el volumen tumoral promedio fue de 1251.97 mm3. Los tumores tuvieron una configuración de domo (50%) y de seta (50%), de tamaño mediano a grande. Tres pacientes tuvieron un tumor T3b y uno tuvo un tumor T4d en fase inicial según el American Joint Committee on Cancer (AJCC). Se prescribió una dosis promedio de radiación de 2763 ± 181,3 cGy al 90% de la línea de isodosis. Todos los pacientes lograron el control local del tumor después de una sesión de radiocirugía en la última visita de seguimiento. Un paciente presentó datos de toxicidad aguda (desprendimiento seroso de retina) y fue tratado con éxito. Ninguno de los pacientes precisó enucleación secundaria. Conclusiones: El abordaje multidisciplinario con CK® es una terapia efectiva en el manejo del MCC-C de tamaño mediano a grande. Es necesario mayor tiempo de seguimiento para confirmar estos hallazgos y evaluar la morbilidad a largo plazo.

Abstract Objective: To report the early results of CyberKnife® (CK®) stereotactic radiosurgery in advanced stages of ciliochoroidal (CBCh) melanoma in Mexican patients. Method: A retrospective review of charts was performed to analyze the outcomes of patients who underwent CK® (Accuray Inc, Sunnyvale, CA, USA). Results: Four patients with CBCh melanoma were treated under this protocol. The mean age was 53.2 ± 5.3 years (range: 47-60). Median of follow-up was 9.5 ± 3.1 months (range: 5-12). Mean tumor diameter was 13.49 mm, mean thickness, 11.74 mm, and mean gross tumor volume, 1251.97 mm3. Tumors were dome- (50%) and mushroom-spahed (50%), in medium-to-large sizes. Three patients had T3b tumors, and 1 had a T4d tumor at the early tumor staging according to the American Joint Committee on Cancer (AJCC). A mean dose of 2763 ± 181.3 cGy was prescribed to the 90% isodose line. All patients achieved local tumor control after a single radiosurgical session at the latest follow-up. One patient presented with acute toxicity (extensive serous retinal detachment associated with radiation induced tumor vasculopathy) that was promptly managed. None of the patients required secondary enucleation. Conclusions: CK® appears to be an effective therapy for the management of medium-to-large sized CBCh melanoma. A prospective comparative study with longer follow-up is needed to confirm these findings and assess long-term morbidity.

RESUMO O melanoma iridociliar em anel é um tipo incomum de melanoma uveal. As manifestações clínicas variam desde casos assintomáticos até síndromes mascaradas que mimetizam um glaucoma refratário. As opções de tratamento incluem radioterapia e enucleação. O manejo do melanoma uveal metastático continua desanimador. Novas terapias usando inibidores de checkpoint imunológico estão atualmente em estudo. Apresentamos o caso de uma mulher hispânica de 54 anos com perda progressiva da visão por um melanoma metastático em anel, com semeadura de câmara anterior, tratada com pembrolizumabe.

ABSTRACT Iridociliary ring melanoma is an uncommon type of uveal melanoma. Clinical manifestation varies from asymptomatic cases to masquerade syndromes mimicking refractory glaucoma. Treatment options include radiotherapy and enucleation. Management of metastatic uveal melanoma remains discouraging. Novel therapies using immune checkpoint inhibitors are currently under study. We present a case of a 54-year-old Hispanic woman with progressive vision loss due to metastatic ring melanoma with anterior chamber seeding treated with pembrolizumab.