Despite the high risk of intraocular surgery, the need to preserve the only potentially seeing eye with treated retinoblastoma may require PPV. Thus, in unclear cases of VH with suspected viable tumor, PPV with intraocular melphalan irrigation, with caution, may be a reasonable procedure.

Отдаленные итоги лечения детей с интраокулярной ретинобластомойЦель -проанализировать отдаленные результаты комбинированного лечения пациентов с интраокулярной ретинобластомой групп C и D. Материал и методы. Всего в исследование включено 70 пациентов (77 глаз) с интраокулярной ретинобластомой (группы C -22 глаза и D -55 глаз), получавших лечение в 2012-2017 гг. по трехстороннему протоколу органосохраняющего лечения ретинобластомы RB2012. У 28 пациентов имела место бинокулярная ретинобластома, у 42 -монокулярная. Средний возраст пациентов с монокулярной ретинобластомой составил 17,1 ± 1,2 месяца, с билатеральной -9,5 ± 0,9 месяца. Средний срок наблюдения -46,0 ± 13,7 (28-77) месяца. Результаты. В 37,6% случаев (n = 29) отсутствовала необходимость в локальной терапии после проведения суперселективной интраартериальной и интравитреальной химиотерапии в связи с полной регрессией опухоли клинически и по данным ультразвукового исследования. Фокальные методы применяли при стабилизации заболевания (n = 23), прогрессировании (n = 8, из них новые очаги опухоли на сетчатке в группе C (n = 1) и D (n = 7)) и рецидиве (n = 14, из них новые очаги на сетчатке в группе C (n = 5) и D (n = 6) и новые очаги на сетчатке и отсевы в стекловидное тело в группе D (n = 3)). В ряде случаев в качестве «долечивания» использовали агрессивные методы органосохраняющего лечения: вторую линию системной химиотерапии (n = 5), дистанционную лучевую терапию (n = 2), стереотаксическую хирургию с применением аппарата «Гамма-нож» (n = 4), а также ликвидационное лечение -энуклеацию (n = 14). Общая пятилетняя выживаемость составила 97,7%, общая пятилетняя органная выживаемость -81,8% в группе C и 80,0% в группе D. Выводы. Показана высокая эффективность комбинированного органосохраняющего лечения интраокулярной ретинобластомы. Общая пятилетняя выживаемость достигла 97,7%, при этом удалось сохранить 80,9% глаз, из них в подавляющем большинстве случаев -без проведения дистанционной лучевой терапии. Как следствие -снижение вероятности развития вторых злокачественных опухолей и повышение качества жизни пациентов.

Introduction. Retinoblastoma (RB) is a malignant tumor of the retina, the survival rate for the intraocular form reaches 99 %. According to the researchers, the risk factors for the occurrence of refractory or recurrent forms of RB are the size of the tumor at primary diagnosis (more than 15 mm), the child's age at the onset of the disease (up to one year), bilateral RB, and the presence of subretinal and vitreal screenings. If methods of local destruction of the tumor are not applicable for the successful treatment of intraocular refractory or recurrent RB, then they resort to the second line of polychemotherapy as an alternative to enucleation. New versions of local chemotherapy and radiation therapy (gamma knife) are being developed and applied. Nowadays, researchers have a negative attitude towards traditional radiation therapy (EBRT) as a method of organ-preserving treatment due to serious consequences (asymmetry of the facial skeleton and second radio-induced tumors).Aim of the study — to increase the effectiveness of organ-preserving treatment in patients with refractory or recurrent intraocular RB.Materials and methods. In the period from 10.2018 to 12.2019 at the N.N. Blokhin National Medical Research Centre of Oncology it was received three-component selective intra-arterial chemotherapy (SIAC) in 15 patients (15 eyes) aged 11 to 52 months. Eyes initially affected by RB (n = 15), according to the IIRC (Murphree) classification, had the following groups: group B in 1 case, group C in 3, group D in 7, and group E in 4 eyes. It should be noted that all patients initially received intensive combined conservative therapy: in 11 (73 %) of 15 cases — systemic chemotherapy VEC; in 13 (86 %) of 15 — SIAC with melphalan, in all cases, additionally, two-component SIAC was used in combination with melphalan with topotecan. In some cases, local therapy in the form of intravitreal chemotherapy (IVIC) was added to the treatment of 6 (40 %) of 15 patients in the form of melphalan monotherapy and in one case (6 %) a doublet was used in combination with melphalan and topotecan. Also, in some patients, additional local ophthalmic therapy was used: transpupillary thermotherapy (TTT) in 6 out of 15 patients, including in combination with cryotherapy (CT) in 1 patient and in combination with brachytherapy (BT) in one case. At the same time, incomplete tumor regression (refractory form of the disease) was noted in 7 out of 15, and a recurrent course of the disease — in 8 out of 15 patients. Incomplete tumor regression and recurrent RB were the reasons for the appointment of a three-component SIAC using carboplatin, melphalan and topotecan, in combination with IVIC with pronounced endophytic growth of RB, as an alternative to irradiation and enucleation of the eye.Results. The average number of three-component SIAC courses was 1.46 ± 0.63 (rang 1—3). The combination of SIAHT with IVHT was performed in 3 out of 15 patients. All children (n = 15) are alive with a median period of follow-up of23.92 ± 15.68 (10—64) months. One in 15 patients was lost from observation due to their parents’ desire to be treated abroad. It is known that the child is alive, but there is no data on the condition of his eyes. 9 out of 14 patients needed additional treatment after SIAC intensification. To consolidate the resulting effect as a full regression in 2 cases, the final IVIC courses were conducted (n = 2). In connection with the presence of residual tumor and/or intraocular progression and/or appearance of new lesions in 1 of 9 patients were IVIC and TTT, 2 of 9 — IVIC, SIAC, CT, TTT. In 4 out of 9 cases, enucleation was performed, and in 2 out of 4 — the operation was performed after continuing the attempt of organ-preserving treatment: after CT and TTT (n = 1) and IVIC, CT, TTT and BT (n = 1). No child was given ERBT. There were no cases of stroke after SIAC. We managed to save 10 (71.4 %) of 14 eyes with visual acuity: object vision in 4 (40 %) out of 10, eye fixation in 3 (30 %) out of 10, and light perception in 3 (30 %) out of 10 cases. The average follow-up period from the end of three-component SIAC in 14 patients was 13.57 ± 5.27 (6—22) months.Conclusion. Thus, the three-component SIAC is effective and safe for patients with refractory and recurrent retinoblastoma, as evidenced by a high percentage of retained eyes and the presence of visual functions from light perception to object vision.

Retinoblastoma is one of highly curable diseases; today the total 5-year survival rate in patients with retinoblastoma exceeds 95%. The article summarizes the current world experience on treatment of patients with intraocular retinoblastoma. The treating skills of intraocular malignant tumor in children are a balance between the patient’s life and the preservation of an eye and its visual functions. The complex and challenging task is the treatment of common intraocular retinoblastoma groups «C», «D», «E» when the large size or localization of the tumor does not allow performing the local (focal) destruction of the tumor. As a rule, in such cases neoadjuvant chemotherapy (CT) is performed at the first stage in order to reduce the size of the tumor for further focal therapy. However, the analysed data on the effectiveness of neoadjuvant CT in combination with focal or radiotherapy demonstrated the limited possibilities of the proposed therapy. Local drug delivery in cancer therapy became a real breakthrough in the organ-preserving treatment of children with large intraocular retinoblastoma. The most widely used current methods of local drug delivery are intravitreal (IVitC) and selective intra-arterial chemotherapy (SIAC) as monotherapy or in combination with neoadjuvant CT and focal therapy which significantly increased the percentage of preserved eyes without radiotherapy administration or damage to the patient survival. The review discusses the different IVitC and SIAC techniques, chemotherapy schemes, dosages of chemotherapy, immediate and long-term complications of treatment.

Introduction. The main reason for enucleation of the eye when attempting organ-preserving treatment are intraocular tumors that do not respond well to chemotherapy.Purpose. The aim of the study was to find new effective and safe organ-preserving methods of treatment of intraocular refractory and recurrent retinoblastoma (RB).Materials and methods. The study included female chinchilla rabbits. The cell line of human RB was intravitreally inserted into the right eye of each animal. The left eye was a control. After obtaining a stable intraocular growth of RB and prolongation of the growth of RB, human cytotoxic lymphocytes (CTL) were intravitreally injected into the affected and control eye K1 at a concentration of 1 × 106 in 0.1 ml suspension and K2 – 5 × 106 in 0.1 ml, respectively.Results. Histological examination of the removed right eyes in animals confirmed the presence of nodal growth of malignant small-cell tumor on the border of the vascular and retinal membranes (epicenter in the choroid) on the posterior wall of the eye. There was scant lymphoid infiltration, without signs of therapeutic pathomorphosis. In the removed animal»s left eyes there is a preservation of histological structures of the eyeball, without dystrophic changes in the cells of the retina and vascular membranes. In the area of limb unit microcap lymphohistiocytic infiltration (with no elements of the tumor). In the choroid single scattered lymphocytes.Conclusion. A short period of observation of the tumor did not allow conclusions about the effectiveness of CTL, but the concentrations of CTL used in the experiment did not lead to dystrophic changes in retinal cells and the choroid of the eye, which is an important factor in overcoming the toxicity of the proposed adoptive immunotherapy. At the next stage of the experiment, in our opinion, it is necessary to study in more detail the cytotoxic effect on healthy structures of the eye and the effectiveness of CTL in a larger number of affected rabbit eyes, using their different concentrations and multiplicity of administration.