Case of April, 2016. This was a 4 yo boy, in whose eye mother had noticed white stuff one week before presentation. Video shows the status of the posterior segment via biomicroscopy. Gross white retinal tumor. Far vitreous tumor seeds. Concurrent wide retinal detachment. B-scan showed intraocular tumor with more than 10 mm base, with vitreous seeds and diffuse calcifications within the tumor.
This retinoblastoma grade is “E” (international retinoblastoma classification, the tumor occupies more than half of the eyeball). The wide spread treatment at the time for group E was (and probably still is) enucleation. Intravenous chemotherapy is mainly indicated in case “high-risk” tumor was confirmed via pathology. However, intraarterial chemotherapy was shown to be effective in eye salvage in some cases (Evidence for Stage-based Chemotherapy, 2015).
In African setup, in Kenya, Tansania, Zambia and other countries of COECSA region, the protocol for retinoblastoma treatment exists. However, the threshold for enucleation is very low, due to the known statistics: in Africa, only 30% of children with retinoblastoma survive. At the same time, it is 97% in the Western world.
The eye was enucleated, and the tumor was not proved to be “high risk”, i.e. no intrusion beyound the eye wall or into the optic nerve section.
The wide spread practice in the World is to implant orbital prosthesis simultaneously after enucleation. This can be not the case in rural African setup, where no skills or prostheses are often available. Postponed eye posthesis is also possible, with worse esthetic and prosthesis function prognosis due to fast developing asymmetry in kids’ orbits and fast conjunctiva contraction in empty socket.
Ретинобластома Группа “Е”. Мальчик 4 лет. Мама заметила белый зрачок неделю назад. Картина при биомикроскопии – на видео. Биомикроскопия: Обширная белая опухоль сетчатки. Отсевы далеко в стекловидное тело. Массивная отслойка сетчатки. По результатм ультразвукового исследования: массивная внутриглазная опухоль с оcнованием более 10 мм, с дальними отсевами в стекловдное тело, с диффузными кальцификатами.
Данная ретинобластома относится к группе E по международной классификации ретнобластом. Лечение опухолей из группы Е – энуклеация. Интраартериальная химиотерапия улучшает прогноз при применении в высокорисковом глазу. Глаз был энуклеирован. Второй глаз – не вовлечён.
В заключении гистологов: внутриглазная опухоль без прорастания в зрительный нерв без вовлечения оболочек, с обширным внутренним некрозом, “маленькие круглые голубые клетки”. Выживаемость при ретинобластоме в странах Африки = 30%, на Западе – до 97%.
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