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Treatment of patients with high-risk neuroblastoma (NB) is a complex challenge, and it is based on response to certain elements of therapy. The development and introduction of new treatment approaches, such as GD2-targeted immunotherapy (IT), leads to improved survival in this cohort of patients. The aim of the study was to retrospectively assess the effectiveness of therapy in patients with high-risk NB before the introduction of IT into clinical practice. We retrospectively analyzed the data of 151 NB patients stratified into a high-risk group who had received treatment in accordance with the modified NB2004 protocol of the German Society for Pediatric Oncology and Hematology (GPOH) at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology from 01.2012 to 12.2017. This study was approved by the Independent Ethics Committee and the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. All the study subjects (or their legal representatives) signed a voluntary informed consent form indicating their agreement to treatment and use of their data for research purposes. Overall survival (OS), event-free survival (EFS), and risk factors were analyzed in the patients with high-risk NB including those who had completed multimodal therapy with autologous hematopoietic stem cell transplantation and post-consolidation therapy with isotretinoin and had achieved a satisfactory response to induction therapy (complete response (CR), very good partial response (VGPR), partial response (PR)) (population of special interest). The main unfavorable prognostic clinical and molecular genetic factors affecting survival in the high-risk NB patients were older age, MYCN gene amplification, and stage 4 of the disease. The use of the modified GPOH NB2004 protocol resulted in a satisfactory response (CR/VGPR/PR) to the induction therapy in most patients: 124/151 (82.1 %). Surgery (other than primary tumor biopsy) led to improved survival, with no statistical difference between macroscopic radical surgery and macroscopic residual tumor. At the same time, radiation therapy (RT), as the second element of local control, had a significant impact on EFS in the group of the patients with stage 4 disease: the 3-year EFS was 39.4 % (95 % confidence interval (CI) 23.1–55.4) in the patients with RT versus 25.7 % (95 % CI 17.5–34.7) in the patients without RT (p = 0.0295). The introduction of a new high-dose TreoMel chemotherapy regimen did not result in worse survival rates but led to a decrease in transplant-related toxicity. The 5-year OS and 5-year EFS were 49.4 % (95 % CI 40.9–57.3 %) and 33.3 % (95 % CI 25.9–40.9) respectively for all the study subjects, and 81.6 % (95 % CI 70.3–88.9) and 55.1 % (95 % CI 43.1–65.5) respectively for the patients from the population of special interest. The analysis of the results of therapy in the high-risk NB patients who had received treatment at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, yielded results comparable to those of the original GPOH NB2004 protocol. The patients with CR/VGPR/PR to the induction therapy who had completed the protocol treatment with autologous hematopoietic stem cell transplantation and isotretinoin post-consolidation therapy demonstrated higher 5-year EFS rates. However, there remains a need to develop more effective treatment regimens for high-risk NB.

Peritoneal sarcomatosis/carcinomatosis is a rare observation in pediatric practice. It occurs, as a rule, with various sarcomas, sarcomatoid tumors, desmoplastic small round cell tumors. Cytoreductive surgery followed by abdominal chemoperfusion has established itself as an effective method of treating such conditions in adult practice and is gaining popularity in pediatric practice. Laparoscopic cytoreduction followed by hyperthermic chemoperfusion used in adult patients has a number of advantages: early recovery after surgery, reduced hospital days, timely initiation of adjuvant chemotherapy, however, the experience of using a minimally invasive technique in children is limited. The article presents the first clinical observation in Russian practice of a 17-year-old patient with embryonal testicular rhabdomyosarcoma and peritoneal sarcomatosis of the abdominal cavity, which underwent laparoscopic cytoreductive surgery with hyperthermic chemoperfusion.

Aim. To analyze the results of liver resection for neoplasms of hepatico-caval confluence without hepatic vein reconstruction in children.Materials and methods. From June 2017 to April 2022, surgical treatment was performed in 5 patients with tumors in hepatico-caval confluence requiring resection of the right hepatic vein and/or median hepatic vein.Results. Two children underwent VII, VIII bisegmentectomy, one of them – laparoscopically. One child underwent laparoscopically assisted IVa, VIII subsegmentectomy. Two children underwent I, IVa, VIII trisegmentectomy with atypical SII, III resection, as well as IVa, VIII, VII trisegmentectomy with I segmentectomy. The median duration of5surgery was 445 min. (315–785), the median blood loss was 400 ml (150–3000). In the postoperative period, complications developed in 2 patients (IIIa and IIIb according to Clavien-Dindo classification), no disturbance of venous outflow from the lower segments of the liver was detected. Vascular margin of R1 resection was obtained in case 1.Conclusion. In the majority of cases of hepatico-caval confluence tumors in children, liver resection, when carefully planned, does not require prosthetics of the right hepatic vein and/or middle hepatic vein and is not accompanied by venous outflow disturbance from the lower liver segments by means of the collaterals to the preserved hepatic veins.

Infectious esophagitis (IE) is commonly seen in immunocompromised patients. IE may be the first symptom of immunodeficiency state, also can be complication of immunosuppressive therapy in patients with hematological and oncological diseases. Severe complication of IE is esophageal stenosis. Patient management tactics continue to be discussed. A purpose of our publication is to demonstrate our experience in the management of patients with infectious esophageal stenosis. The etiology, features of the clinical and endoscopic picture, as well as the effectiveness of drug therapy and endoscopic methods of treatment have been analyzed. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. In each case, parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications.

Cystic nephroma (CN) is a rare renal tumor occurring in children which belongs to a group of neoplasms linked with the inherited DICER1 syndrome. Given the rarity of CNs, it is important to describe clinical, radiological, and molecular genetic characteristics of these tumors in children and adolescents as well as to analyze treatment outcomes. We present our experience in managing 8 patients with histologically verified CN who received treatment and consultations at the D. Rogachev NMRCPHOI over a period of 9 years (2012–2020). The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. The patients’ parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications. We performed a retrospective analysis of clinical presentation, radiological findings, the extent of treatment given to patients, treatment outcomes, and the results of molecular genetic testing. The study included patients aged between 8.6 and 197 months at diagnosis (the median age was 14.2 months). The analysis of initial complaints revealed that six patients (75%) had an increased abdominal girth and a palpable mass in the abdomen, one patient (12.5%) presented with arterial hypertension, and another patient (12.5%) had a mass detected by a routine abdominal ultrasound examination. On contrast-enhanced computed tomography scans, CNs appeared as multicystic masses with thin, contrast-enhancing septa; the CN volume ranged from 59.7 to 1293.1 cm3 (the median volume was 626.3 cm3 ). In all cases, the diagnosis of CN was verified histologically. Surgical treatment included nephrectomy (n = 6) or partial resection of the affected kidney (n = 2) with the removal of the tumor. Some patients (n = 5) included in our analysis received pre-operative chemotherapy at the discretion of their treating physicians. Molecular genetic testing was carried out for 7 children: 4 out of 7 patients (57.1%) were found to have somatic and germline mutations in the DICER1 gene. Carriers of pathogenic DICER1 variant were identified in the family of 1 patient. The median duration of follow-up was 17.6 months (range: 1.7 to 58.9 months). Currently, all patients are alive, no relapses have occurred. Cystic renal neoplasms detected by radiological investigations should be reviewed at the reference centers for pediatric oncological diseases and included CN in the differential diagnosis. Initial surgery is the first line of treatment for cystic nephroma. The final diagnosis is made on the basis of a histological examination of tumor tissue. All patients with confirmed CN should be referred for genetic counseling and molecular genetic testing for germline mutations in the DICER1 gene and should receive surveillance recommendations for the early detection of other metachronous DICER1-associated tumors.

Clear cell sarcoma of the kidney (CCSK) is a rare malignant renal tumor in children, which accounts for 2–5% of pediatric kidney malignancies. The aim of the study was to analyze the results of therapy of patients with CCSK treated in Dmitry Rogachev National Medical Research Center оf Pediatric Hematology, Oncology and Immunology. Retrospective analysis of patients with a histologically confirmed diagnosis of CCSK treated for the period 01.2012–02.2020 (98 months) was done. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI of the Ministry of Healthcare of the Russian Federation. Demographic characteristics, clinical symptoms, methods of diagnosis, and treatment modalities were analyzed. Patients were treated according to the protocols of the SIOP-RTSG group (SIOP 93-01, SIOP-2001, SIOP-RTSG-2016). The stage was assigned according to the SIOP classification. Overall and event-free survival was assessed by the Kaplan–Mayer method. The analysis of the results was carried out on 01.03.2021. The analysis included 10 patients with CCSK. The median age at the time of diagnosis of CCSK was 30.1 months (range 13.5–70.8 months). All patients were male. The duration from the onset of the first symptoms/detection of the tumor to the diagnosis was 0.8 months (range 0.1–3.2 months). The diagnosis was established on the basis of clinical and radiological data (n = 9) and biopsy (n = 1). Distant metastases at the time of diagnosis were detected in 1 (10%) patient (localization of metastases - lungs). The median tumor volume was 439 cm3 (range 256–996 cm3 ). Preoperative chemotherapy was performed in all patients (AV regimen (actinomycin D, doxorubicin) in 7 (70%) patients). Assessment of response after preoperative chemotherapy showed tumor regression in 3/10 (in 1/7 with AV regimen), tumor progression in 5 and stable disease in 2 patients. Surgical treatment in the extent of nephrectomy was performed in all patients. In 1 (10%) case, intraoperative tumor rupture was documented. Distribution of patients by local stages: I – 4/10 (40%), II – 2/10 (20%), III – 4/10 (40%) (including 1 patient with distant metastases). In 1 patient, a left thoracotomy was performed to exclude lung metastases. Adjuvant chemotherapy was performed in all patients in accordance with the relevant protocols of the high-risk group: 7 – 4–5-drug regimen, 3 – AVD regimen (actinomycin D, vincristine, doxorubicin). Radiation therapy was performed in 6/10 (60%) patients. Outcomes: 9/10 (90%) – alive, 1/10 (10%) patient died (non-tumor-related death). 3-year event-free survival and overall survival were 78.8% (95% confidence interval (CI) 52.5–100) and 90.0% (95% CI 71.4–100) respectively. Intensive program therapy in patients with CCSK allows to achieve satisfactory results of treatment.

The incidence of posthepatectomy liver failure in adult patients and a large number of complications of two-stage liver resections require a search for criteria that allow highly accurate assessment of the risk of liver failure. For this purpose, the study of the future liver remnant volume and function have been widely introduced among adult patients, and the future liver remnant function measurement reflects the greater sensitivity. The absence of references to posthepatectomy liver failure, as well as the experience of determining the functional reserve of the future liver remnant in children, let us to suggest the possibility of a wider using one-stage liver resections when the future liver remnant volume is below the generally accepted threshold (25% of the healthy liver parenchyma volume) in the case of the functional reserve sufficient value. This clinical case describes the successful extended right hemihepatectomy and segmentectomy 1 in a 3-year patient with a future liver remnant volume of 16.5% without clinical signs of postresection hepatic failure, which confirms the thesis of the need to assess the functional liver reserve in pediatric oncology to reduce the frequency of two-stage resections and liver transplants. The patient’s parents gave consent to the use of their child’s data, including photographs, for research purposes and in publications.

Osteosarcoma (OS) is the most common primary bone tumor in children and adults. In 15–20% of patients, distant metastases are detected at the time of diagnosis of OS. In more than 80% of cases, metastases are located in the lungs and are the most common disease-related cause of death in OS patients. OS can only be cured if complete surgical remission (CSR) in the lungs is achieved through surgery involving palpation, identification and resection of all detected metastases. Among thoracic surgeons, it is common practice to perform wedge resection of the affected lung parenchyma as it spares more healthy lung tissue. Lobectomy or pneumonectomy can be carried out if either is indicated in the patient. There is, however, no consensus on the best surgical approach for metastasectomy. Our study includes 24 patients who underwent simultaneous bilateral thoracotomy at the Department of Oncology and Pediatric Surgery of the D. Rogachev NMRCPHOI in the period from February 2018 to May 2021. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. Eighteen patients underwent primary surgery as part of combination protocol treatment, and six patients were surgically treated for relapse. In 66.7% of the patients treated with upfront surgery, the number of lesions was underestimated, as evident from computed tomography images and intraoperative findings. Post-treatment necrosis grade IV was detected only in 3 patients, in 21.1% of the resected metastases. The median time from bilateral thoracotomy to systemic anti-cancer therapy reinitiation was 12 days. Two patients experienced progression of metastatic disease in the lungs during and immediately the protocol treatment. At the last follow-up, 3 patients were alive with evidence of disease, and 2 patients had died of OS progression. A total of 33.3% of the patients who had had primary surgery developed metastatic (n = 6) and local (n = 1) relapses.

Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) is treatment of choice for determined group of adult patients. Cytoreduction and HIPEC is usually used in patients with ovarian, gastric and colorectal cancer, appednix cancer, peritoneal mesothelioma and in some tumors of another localization. The experience of cytoreductive surgery and HIPEC in childhood is limited due to the lower frequency of peritoneal carcinomatosis in pediatric population. However, about 30% of malignant tumors in children develop in peritoneal cavity and pelvis. High recurrence rate are typical for these tumors, particularly due to tumor rupture. Taking into consideration low frequency of epithelioid tumors in children HIPEC is used for patients with other tumor types such as desmoplastic small round cell tumor (DSRCT), germ cell tumors, rhabdomyosarcoma and others. The aim o this paper is the review of the literature and present experience analysis of cytoreductive surgery and HIPEC in children.

s of Current Foreign Publications.

The authors have analyzed the indications and outcomes after ALPPS procedure considering modern literature data devoted to this surgery in pediatric patients. No data on post-resection liver failure, as well as unclear future liver remnant function make it possible to question the feasibility of such procedures in children.На основании изучения существующих публикаций, посвященных операции associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) в педиатрической популяции пациентов, проведен анализ показаний и результатов вмешательств. Отсутствие данных о развитии пострезекционной печеночной недостаточности, а также неизученный функциональный резерв будущего остатка печени позволяет поставить под сомнение целесообразность подобных операций у детей.

The article presents clinical case of successful treatment of patients with metachronous, resectable liver metastasis of colorectal cancer located in hepatic veins confluence. It is shown the results of consecutive chemotherapy and portal vein embolization which reduced metastasis size and increased the volume of the left liver lateral sector. Readiness for transplantation has allowed to perform radical R0-resection. We obtained satisfactory long-term outcomes.

The study was carried out to investigate etiology and rate of prevalence of nosocomial strains of microbes in biosubstrates of patients in case of development of infectious complications and level of their resistance to various groups of antibiotics. The retrospective analysis of agents of post-operational infectious complications and their sensitivity to antibiotics in 99 patients after operations of transplantation of liver and kidneys in 2013. The patients were allocated on three groups: group I - patients of department of reanimation and intensive therapy after mentioned operations (17 individuals); group II (42 individuals) - after orthotopic transplantation of liver (19 patients) and orthotopic transplantation of kidneys (23 patients); group III (57 patients) - after kindred transplantation of hepatic lobe from living relative donor. The retrospective analysis of agents of post-operational infectious complications in patients underwent transplantation of liver and kidneys demonstrated that out of 1285 identified strains of microbes, 60.8% were presented by Gram-positive microflora with predominance of coagulase-negative staphylococci and enterococci. Among Gram-negative microflora, enterobacteria comprised the largest percentage (61%). The analysis of antibioticgrams of agents of infectious complications established low level of sensitivity of strains of both Gram-positive and Gram-negative microbes isolated from patients of department of reanimation and intensive therapy and to carbapenems and average level of sensitivity to Tigacil. In overall, poly-resistance of strains was established. The differences in antibiotics resistance for strains of pathogens isolated in department of reanimation and intensive therapy and specialized departments were established. The received data can be used for correction of application of antibiotics in prevention and therapy of post-operational infectious complications.

Liver transplantation is a life-saving procedure for many forms of end-stage liver disease in pediatrics. Cytomegalovirus (CMV) is the most common and signifi cant posttransplant infection after pediatric liver transplant (PLT) with developing an episode of CMV infection or disease. It is well known that CMV increases risk of graft loss. The review presents aspects of etiology and epidemiology of CMV after PLT, approaches employed in diagnostics and prophylaxis of CMV, algorithms for valganciclovir dosing and methods to prevent complications associated with CMV. The latest data on current prevention strategies in pediatric liver transplantation centers in the world are also presented.