Currently a priority in children oncology is a limb salvage treatment. Achieved in recent years advances in chemotherapy of malignant bone tumors, improved surgical techniques, and also introduction in orthopedics of the newest technologies allow to considerably expand the indications for endoprosthesis. A feature in cases in children of early age is the use of sliding implants type non-invasive and minimally invasive that subsequently allows gradual correction of leg length and avoid the various disorders of musculoskeletal system (shortened limbs, spinal deformity), thereby improving the quality of life of patients. In some cases, for example, when a tumor is localized in the upper limb region, the optimal solution may be the use of autologous grafts on microvascular anastomoses. In the case of tumor localization in the pelvic region, the use of implants made by means of 3D-modeling, in our opinion, is also an optimal solution.
Меланома кожи у детей встречается очень редко и по ряду характеристик отличается от меланомы кожи у взрослых. Ранняя диагностика этой высокозлокачественной опухоли является главным условием успешного лечения. При определении заболевания у детей обычно используют те же методы, что и у взрослых. Адекватность такого подхода требует проверки. Цель исследования-описание процедур постановки диагноза для крупного врожденного пигментного новообразования кожи у ребенка. Описание клинического случая врожденной опухоли в области лучезапястного сустава у девочки 5 мес. По месту жительства было высказано предположение о гемангиоме. При диагностике в НИИ детской онкологии и гематологии НМИЦ онкологии им. Н. Н. Блохина проведены ультразвуковое исследование, магнитно-резонансная томография, трепанобиопсия, открытая биопсия. Материалы последней исследованы гистологически на препаратах, окрашенных гематоксилином и эозином, и иммуногистохимически путем детекции индикаторов пролиферации клеток, антигенов меланоцитарного ряда дифференцировки, белков, используемых как маркеры собственно меланомы, антигенов тканевой совместимости. Сделано заключение: узловая пигментная меланома кожи. Проведено иссечение опухоли. Безрецидивный период на момент подготовки сообщения-12 мес. Заключение. Описаны процедуры диагностики и лечения 5-месячной девочки с врожденным пигментным новообразованием кожи в области лучезапястного сустава. В процессе диагностики использованы подходы, применяемые при выявлении более частой и лучше изученной меланомы кожи у взрослых. Для подтверждения адекватности использованных в работе методов в диагностике пигментных новообразований кожи у детей целесообразно продолжение исследований.
Ewing's sarcoma (ES) is a highly malignant tumor of children and adolescents that affects bones and soft tissues with the same frequency. The origins of ES are the subject to many discussions. Differential diagnosis is complicated and requires a full range of immunohistochemical and molecular genetic studies. The prognostic value for extraskeletal and bone ES under current chemotherapy protocols is unknown and requires further analysis. The purposes of this research were a comparative analysis of clinical characteristics, therapeutic approaches and outcomes of the disease in patients with extraskeletal and bone localizations of ES. Materials and methods used: a single-center retrospective cohort study was conducted, which included 330 patients (237 (71.8%) boys/93 (28.2%) girls) aged 0 to 18 y/o (median 11 [7; 14] y/o) with confirmed diagnosis of ES who received treatment in 2008-2022, of which 280 (84.85%) with primary bone localization (group of bone ES - BES), and 50 (15.15%) with soft tissue localization (group of extraskeletal ES - ESES). Comparative analysis of survival rates for primary tumor localization in the area of bones and soft tissues was performed. The median follow-up time for all patients was 35.5 [18.2; 68.5] months, 37.0 [18.0; 71.0] months with BES, and 29.5 [16.8; 65.5] months with ESES. All patients received treatment according to the protocols adopted at the Research Institute of Pediatric Oncology and Hematology named after Academician L.A. Durnov with the N.N. Blokhin Russian Cancer Research Center (Moscow, Russia): MMES-99, ES-2017. Overall survival (OS) was calculated with the Kaplan-Meier estimator. Results: the selected groups differed statistically significantly by gender (74% of boys in the BES group, and 60% of boys the ESES group, p=0.035) and age (10.5 [8; 15] years in the BES group, and 8.5 [4; 12] y/o in the ESES group, p=0.001). BES was diagnosed statistically significantly more often in older age groups than ESES (p=0.004). Compared with BES, in ESES the tumor was statistically significantly more often located in the region of the axial skeleton and visceral organs (24.0% vs. 56%, p<0.001). Disseminated form of the disease in the BES group was recorded in 110 (39.3%) patients, and in 15 (30.0%) in the ESES group. Authors did not find statistically significant differences in overall 5-year OS for localized forms of BES and ESES (79% and 78.5%, respectively), the median OS in these groups was not reached. The OS of patients with disseminated stages of BES and ESES was statistically significantly lower than in the group of localized forms. At the same time, the 5-year OS was 41.2% and 40.6%, the median OS was 46.9 and 28.4 months (p=0.001, respectively). Differences in 5-year progression-free survival (PFS) for localized forms were 71.6% for BES and 75.6% for ESES (p=0.001), for disseminated forms - 32.4% vs. 44.9% (p=0.036, respectively). In the disseminated stage of BES, progression/relapse was detected in 50% of cases after 21.1 months, and after 20.3 months for ESES. Conclusions: Authors have identified the following unfavorable prognostic factors. As for BES these were: older age, disease stage, primary tumor volume over 200 cm3 and length over 8 cm, radiation therapy in the treatment program or combined treatment. As for ESES, the unfavorable PFS factors were the stage of the disease, the volume of the primary tumor and the process dissemination. In this regard additional analysis of key genomic characteristics is required in order to further determine the risk group stratification and prognosis.
Остеосаркома -крайне злокачественная мезенхимальная, высокодифференцированная опухоль из костной ткани, протекающая агрессивно, характеризующаяся быстрым развитием удаленных метастазов. Саркома Юингане менее сложное заболевание, что объясняется его биологическими особенностями, в том числе агрессивным течением, склонностью к развитию ранних гематогенных метастазов и частых рецидивов. Цель исследования -проверка возможности возникновения осложнений и уровня восстановления функций по шкалам Toronto Extremity Salvage Score (TESS) и Musculoskeletal Tumor Society (МSTS) после эндопротезирования и установки спейсера. Материалы и методы. Нами были рассмотрены клинические случаи 7 детей в возрасте от 8 до 15 лет, получавших лечение в НИИ ДОИГ ФГБУ «Научный медицинский исследовательский центр онкологии им Н.Н. Блохина» Минздрава России в 2013-2019 гг. У 4 пациентов был установлен диагноз остеосаркомы, у 3 -саркомы Юинга. Им было проведено органосохраняющее хирургическое лечение верхней конечности одним из 3 способов: эндопротезирование, установка спейсера и замещение аутокостью на микрососудистых анастомозах. В ходе исследования использовалась методика оценки функции конечности по шкалам TESS и МSTS. Результаты. Было выявлено, что в послеоперационный период у всех пациентов вне зависимости от примененной методики органосохраняющей операции результаты варьировались в пределах 1,4-1,5 по шкале TESS, и 91-95 % по шкале MSTS. У 4 пациентов выявлены послеоперационные осложнения в виде посттравматической нейропатии и вывиха. У 1 больного был диагностирован местный рецидив, у 1 -метастатическое поражение легочной ткани. Следует отметить, что состояние всех пациентов на данный момент удовлетворительное, летальных случаев выявлено не было. Заключение. Мы считаем, что результаты проведенного исследования многообещающие. Однако необходимо осуществить более длительное наблюдение за пациентами и оценить их 5-летнюю выживаемость.
Introduction. Cancer is the second leading cause of death in the world. WHO predicts that the incidence of cancer will grow from 14 million in 2012 to 22 million in the following decades. It is impossible to achieve an adequate effect in the medicamentous treatment of malignant tumors without the use of accompanying therapy. The search and use of new methods of correction of complications arising in children during polychemotherapy will not only signifi cantly reduce the lethality of this category of patients, but also signifi cantly intensify specifi c therapy and thereby increase the percentage of complete remissions and event-free survivals.Goal of research — to assess the effectiveness of osteopathic treatment in eliminating the side effects of adjuvant PCT in children in the postoperative period.Materials and methods. 42 children aged 6–16 years with a history of malignant neoplasm of the musculoskeletal system took part in the research. At the time of the study they were undergoing adjuvant PCT after the radical surgery. Patients were divided into two equal and comparable in all parameters groups (the main group and the control one) by method of blind selection. Patients of the experimental group (21 patients) underwent osteopathic correction according to the standard protocol with the frequency 1 session per 7–14 days. In order to assess the severity of PCT complications, a number of non-specifi c indicators from the toxicity criteria proposed by the International Society of Pediatric Oncologists (laboratory markers of hematological toxicity and clinical manifestations of gastrointestinal toxicity) were used. The osteopathic status was assessed in accordance with the recommendations «Osteopathic Diagnostics of Somatic Dysfunctions» with limitations in active tests because of the severity of the condition and postural problems related to the short time period after surgical treatment (tumor resection, endoprosthetics). The indices for each PCT course were calculated separately. Patients were included in the study during the start of the next course of PCT. The duration of each patient′s observation was at least 21 days, which corresponds to the time between two subsequent courses of PCT.Results. In this group of patients, osteopathic correction led to the elimination of global biomechanical dysfunctions, a decrease in the absolute number of global and regional somatic dysfunctions, and a change in the structure of regional dysfunctions. It has been established that osteopathic support does not signifi cantly affect the intensity of hematological toxicity in PCT (the severity of leukocytopenia, anemia, thrombocytopenia). However, it leads to a signifi cant decrease in such manifestations of gastroenterological toxicity as emetogenic syndrome and abdominal pain.Conclusion. The reasonable use of individual osteopathic correction in the course of accompanying therapy can reduce the intensity of intoxication and improve the quality of life of patients during PCT courses.
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