Individualized therapy in neuroblastoma

Simon, T. Abrams; Fischer, Matthias; Hero, B

doi:10.17650/2311-1267-2016-3-4-36-47

Cited by 3 publications

(2 citation statements)

References 48 publications

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“…спешное лечение детей, страдающих нейробластомой (НБ), на современном этапе может быть обеспечено лишь благодаря строгому соблюдению принципа риск-адаптированной терапии. Своевременная и детальная стратификация пациентов позволяет с успехом применять интенсивную комбинированную терапию для повышения эффективности лечения у одних больных и избегать неоправданно агрессивной тактики у других [1,2]. Так, большим количеством исследований обоснована целесообразность формирования группы наблюдения при отсутствии неблагоприятных прогностических факторов.…”

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“…Факторы, имеющие прямую взаимосвязь с неблагоприятным прогнозом, -возраст пациента на момент диагностики заболевания, стадия, содержание нейронспецифической энолазы (НСЕ). Доказано снижение общей и бессобытийной выживаемости при выявлении амплификации MYCN, хромосомных аберраций 1p и 11q, а также структурных изменений в других генах [1,2,8,10]. В ряде исследований определена неблагоприятная роль первичного метастазирования в легкие [9], кости [6], полиплоидности хромосом в клетках опухоли [10].…”

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Тhe role of surgery in treatment of patients with neuroblastoma of difficult anatomical localization

Сухов

Sokolov

Narbutov

et al. 2019

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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High risk of life threatening complications is distinctive for surgery of tumors, which are in contact with large main vessels. Planning for the removal of the primary focus of neuroblastoma (NB), characterized by similar localization, includes determining the timing and method of the operation, the required resection volume, predicting complications, developing ways to prevent them and relieve them. The study was approved by the Independent Ethics Committee and Scientific Board of N.I. Pirogova of RussianNationalResearchMedicalUniversity. The results of complex treatment of 11 children with NB of thoracoabdominal localization, aged from 9 to 55 months, are present in the research. 7 (64%) of them were stratified into a high-risk group, 3 (27%) – intermediate, 1 (9%) – low, according to the criteria of the NB-2004 protocol. The results were analyzed depending on the features of the operation and the course of the early postoperative period. The number of variants of tumor syntropy which coincided image-defined risk factors, revealed by computed tomography with contrast enhancement, was in the range from 2 to 7 (median – 5). The median volume of the removed part of the tumor was 95% (range from 92 to 98%). Among intraoperative complications aortic wall (1 (9%) observation), superior mesenteric vein (1 (9%) observation), right renal vein (2 (18%) observations), left renal vein (2 (18%) observations), inferior vena cava (2 (18%) observations) injury should be noted, which were sutured without subsequently detected hemodynamic disturbances and organ function. Complications in the early postoperative period were: partial ileus (1 (9%) observation), renal artery thrombosis (1 (9%) observation), inferior vena cava thrombosis (1 (9%) observation), pancreatic necrosis (1 (9%) observation). They demanded reoperation in two children: nephrectomy in a child with renal artery thrombosis at the fourth posroparative day and performing of anastomosis between the pancreas and small intestine at the 74 posroparative day in a patient with pancreatic necrosis. Among patients in the intermediate and high-risk groups, the event-free two-year survival rate was 50%, the total two-year survival rate was 88%. The prognosis of the disease does not reliably correlate with the duration of the relief of postoperative complications (p = 0.53) and the resection volume (p = 0.46). Surgical intervention and postoperative observation in children with NB of thoracoabdominal localization should be carried out by a team that has experience of similar operations, owning vascular suture technique, with a preliminary assessment of the image-defined risk factors. The purpose of the operation should be a resection aimed at cytoreduction and elimination of the mass-effect, without striving to remove all areas of the tumor.

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Тhe role of surgery in treatment of patients with neuroblastoma of difficult anatomical localization

Сухов

Sokolov

Narbutov

et al. 2019

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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Molecular genetic traits and risk factors for neuroblastoma

Klimenko,

Melnikov,

Kulyova

et al. 2023

Pediatr (SPb)

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Neuroblastoma is the most common extracranial solid tumor in childhood, accounting for 815% of all malignant neoplasms in children. The uniqueness of the neuroblastoma etiology and pathogenesis creates significant difficulties for doctors due to the unpredictability of the course of the disease: the tumor can regress and lead to death in a short time, showing significant immunity to ongoing therapy. Early diagnosis of a tumor is necessary to understand the risk factors, the etiology, the mechanisms of neuroblastoma occurrence and further development. To date, a number of different risk factors have been identified, related both to genetic aspects and to aspects related to the course of pregnancy, birth and lifestyle of the childs parents. The list of these risk factors is still incomplete and research to identify new factors continues to this day. Morphological and molecular genetic diagnostics play an important role in this. Undifferentiated and low-differentiated variants of neuroblastoma are often associated with MYCN amplification and other chromosomal aberrations determined by molecular genetic research. Chromosomal instability plays an important role in the process of early tumor development, which probably causes the possibility of acquiring certain genetic changes, which also determines the prognosis and further therapeutic options. In this article, we have identified the most significant risk factors by comparing the results of various studies.

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Surgical Treatment of Children With Neuroblastoma of Complex Anatomical Localization

Сухов

Sokolov

Narbutov

et al. 2018

Russian Journal of Pediatric Surgery

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Introduction. Neuroblastomas arise in the case of intimate association of the tumor with large vessels and internal organs. The early detailed stratification of cancer risk, as well as forecasting possible complications of surgery based on visual diagnostic methods, contribute to the improving the results in this category of patients. Material and methods. The study analyzes the results of the diagnosis and treatment of neuroblastoma of thoracoabdominal localization in 9 children aged from 9 to 55 months. All of them received the treatment according to the NB-2004 protocol. Before surgical intervention, the factors of the surgical risk were estimated from computed tomography with contrast, the number of factors averaged of 5. Results. The inclusion of the left renal artery and aorta into the tumor was revealed intraoperatively in 8 children, the involvement of the left renal vein, the right renal artery, the superior mesenteric artery was found in 7 patients, the right renal vein, the celiac trunk - in 6 cases, and the other large vessels - in a smaller number of cases. Damages of the right renal vein in 2 patients, left renal vein - in 2 children, aorta - in 1 child, inferior vena cava - in 2 observations and celiac trunk in 1 patient was sutured without subsequent complications. After injury and suturing of the inferior vena cava in a 1 patient, there was revealed a parietal thrombus without clinically significant hemodynamic disturbances. In 1 observation, postoperative thrombosis of the left renal artery caused the deterioration of the blood flow requiring nephrectomy. Damage to the pancreas in 1 child was accompanied by a long drainage and the formation of pancreatojejunostomy. The volume of the resection of the tumor amounted on average of 93%. The total number of complications in the early postoperative period accounted for 44%, out of which repeated surgical interventions were required in case of bleeding against the therapy of renal artery thrombosis in 1 patient and focal pancreatic necrosis in the 1 patient. The duration of postoperative follow-up was of 18 months. Overall survival is 100%, the event-free survival rate is of 78%. The continued growth of the tumor was registered in 2 patients in the high-risk group. Discussion. Neoadjuvant chemotherapy contributed to the enhancement of the radicalization of the surgical treatment, leading to a decrease in the size of the primary focus, metastases, and involvement of large vessels and internal organs in the tumor. The radical removal of neuroblastoma is acceptable in the absence of the invasive growth in the walls of large vessels and internal organs, otherwise, the resection should be performed for the purpose of cytoreduction and prevention of organ failure due to tumor compression.

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Individualized therapy in neuroblastoma

Cited by 3 publications

References 48 publications

Тhe role of surgery in treatment of patients with neuroblastoma of difficult anatomical localization

Тhe role of surgery in treatment of patients with neuroblastoma of difficult anatomical localization

Molecular genetic traits and risk factors for neuroblastoma

Surgical Treatment of Children With Neuroblastoma of Complex Anatomical Localization

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