Background: Central liver segments resection (CLSR) still is not widely used in pediatric surgery due to its technical difficulty, whereas this procedure is widely spread as a parenchyma sparing approach of centrally located liver tumors in adults. The aim of this study is to analyze the outcomes of CLSR in comparison with extended hepatectomy (EH) in children with different liver tumors. Methods: A single-center retrospective analysis of patients who received CLSR (n = 14) and EH (n = 44) from June 2017 to December 2023 was applied. Patient's characteristics, preoperative, intra- and postoperative data were compared between 2 groups. Results: Preoperative CT-volumetry showed that future liver remnant volume was higher in CLSR group compared to EH (FLR-V; (54 +/- 29 (40-91) % vs 40 +/- 12 (17-73) %, p = 0.016). The intraoperative blood loss (200 [90-1150] (20-3000) ml vs 100 [30-275] (10-9000) ml, p = 0.088) and transfusion volume (310 [85-590] (0-1860) ml vs 150 [0-310] (0-4770) ml, p = 0.484) were similar in both groups, while operation time was longer in CLSR group (420 [320-595] (145-785) min vs 280 [203-390] (125-710) min), p = 0.011). There was no difference in biliary leakage (3 (21.4 %) vs 12 (27.3 %); p = 0.479), other complications (4(28.6 %) vs 5(11.4 %), p = 0.198) and complications > IIIb by Clavien-Dindo (2(14.3 %) vs 8 (18.2 %), p = 0.385) postoperatively. Conclusion: CLSRs allow to preserve more healthy liver parenchyma compared to EH with similar intraoperative and postoperative outcomes. << Extended mesohepatectomy >> allows to achieve R0 resection when central liver tumor extends on the left lateral and/or right posterior section. Type of Study: Retrospective Comparative Study (Level of Evidence III). (c) 2024 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.

Congenital mesoblastic nephroma (CMN) is a rare renal tumor of young children with intermediate biological behavior, accounting for 3.5-4% of all renal tumors in children. СMN is characterized by a favorable prognosis in case of radical surgical treatment. Relapses of CMN are considered to be quite a rare occurrence (4% of all cases), however, both local and metastatic relapses are possible. There are no fully standardized treatment approaches for patients with relapsed CMN. In our study, we performed a retrospective analysis of patients (n = 3) with a verified relapse of CMN who had received treatment at the D. Rogachev NMRCPHOI between 2012 to 2022 (132 months). At relapse, all the patients underwent at least one part of treatment at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. The diagnosis of CMN was established at the Pathology Department of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation, based on a histological examination. The patients with the cellular histological subtype of CMN underwent fluorescent in situ hybridization testing for ETV6 gene rearrangements. The demographic characteristics, clinical data, the extent of initial treatment and relapse therapy were assessed. Here, we describe three clinical cases of relapse in patients with CMN. The median age at initial diagnosis was 0.8 months (range 0.7–1.4). Our analysis of the extent of primary surgical treatment, including nephrectomy, in all cases revealed the following factors associated with a higher risk of relapse: preoperative tumor rupture – 1, intraoperative tumor rupture – 1, inability to confirm tumor-free margins – 1. The distribution of histological subtypes was as following: classical CMN (n = 1), cellular CMN (n = 1), and mixed CMN (n = 1). One patient had local stage II and 2 patients had local stage III. The median time from diagnosis to disease relapse was 0.8 months (range 2.3–4.3). One patient with mixed CMN died 10.6 months after diagnosis from complications of intensive therapy carried out for extremely aggressive relapse. Two patients are alive after repeated surgical treatment (R1 resection) and adjuvant therapy with actinomycin D and vincristine (AV regimen) for 27 weeks in one case, and neoadjuvant therapy (AV regimen for 4 weeks), delayed surgery (R0 resection), and adjuvant therapy (AV regimen for 4 weeks) in the other case. These patients were followed up for 92.2 and 21.3 months, respectively. By acknowledging the possibility of recurrent CMN, it seems important to provide multidisciplinary clinical care to young children with renal tumors involving a detailed planning of surgical procedures, radical surgeries in accordance with practice guidelines and standards in surgical oncology, and careful follow-up, especially during the first year after surgery. The patients' parents gave consent to the use of their children's data, including photographs, for research purposes and in publications.

Metanephric tumors (MTs) are a group of rare childhood kidney tumors consisting of epithelial and/or stromal cellular elements and characterized by a variety of histopathological features. MTs include metanephric adenoma (MA), metanephric adenofibroma (MAF), and metanephric stromal tumor (MST). This study aimed to retrospectively analyse clinical and molecular genetic characteristics of MTs, verified at the Pathology Department of the Dmitry Rogachev NMRCPHOI of Ministry of Healthcare of the Russian Federation. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. We performed a retrospective analysis of data on patients with MTs histologically confirmed between February 2015 and February 2024 (109 months). During this period, a total of 26 cases (patients aged 0–18 years) of histologically confirmed MA, MAF, and MST had been documented at the Pathology Department of the D. Rogachev Center. Out of these 26 cases, 16 patients with known clinical data were included in our analysis. The median age at diagnosis was 3.5 years (range: 0.4–15.2 years). The boys:girls ratio was 1:1. The analysis of primary complaints showed that the majority of patients (n = 8; 50%) were asymptomatic and their kidney masses were detected by chance. The rest of the patients presented with pain syndrome (n = 3; 19%), gross hematuria (n = 2; 13%), intoxication syndrome (n = 1; 6%), polycythemia (n = 1; 6%), an increased abdominal circumference (n = 1; 6%). Primary surgery was performed in 6 (37%) patients: partial nephrectomy (n = 4), total nephrectomy (n = 1), and a core needle biopsy of the mass followed by partial nephrectomy (n = 1). Ten patients (63%) underwent preoperative multiagent chemotherapy. The analysis of the extent of surgical treatment of all patients included in the analysis (n = 16) showed that total nephrectomy was performed in 9 cases, and partial nephrectomy – in 7 cases. R0 resection was achieved in 15 cases, R1 resection – in 1 case. The distribution by histological variants was as follows: MA – 10 (63%) patients, MST – 3 (19%) patients, MAF – 1 (6%) patient, MA in combination with clear cell papillary renal cell carcinoma – 1 (6%) patient, MAF in combination with papillary renal cell carcinoma – 1 (6%) patient. Sixteen patients underwent molecular genetic testing: a somatic V600E mutation in the BRAF gene was detected in 10/16 (62.5%) patients. Currently, all patients are alive, and no relapses of the disease have been observed. MTs are a group of rare kidney tumors in children, characterized by a variety of histological patterns, which creates difficulties in differential diagnosis with other kidney tumors, such as renal cell carcinoma and nephroblastoma. Molecular genetic testing aimed at identifying mutations in the BRAF gene can help in establishing the correct morphological diagnosis.

Aim. To carry out a retrospective analysis of the treatment outcomes of the patients who underwent liver resection with angioplasty facilitated by transplantation techniques. Materials and methods. The sample of cases was divided into four groups: liver resection with total vascular isolation of the liver, ante situm liver resection, hepatic artery and portal vein reconstruction, cardiopulmonary bypass resection. The analysis was focused on surgery features and treatment outcomes. Results. The study involved 29 patients. The performed interventions included 11 liver resections with total vascular isolation, 6 ante situm liver resections, 7 resections with angioplasty, and 5 cardiopulmonary bypass resections. 5 patients (17 %) developed complications that required reoperation. Within 90 days, 4 patients (14 %) died. For patients with malignant neoplasms, one-, three-, and five-year overall survival rates accounted for 84.4 %, 58.4 %, and 51.1 %; one-year event-free survival rate comprised 57.1 %, and three- and five-year event-free survival was 41.7 %.The study revealed no significant differences in the complication rates. The groups of ante situm liver resections and cardiopulmonary bypass resections significantly differed from the other groups by the higher incidence of tumor progression, invasion of adjacent organs, hemorrhagic complications, preoperative stay, and duration of cold perfusion of the liver. The cardiopulmonary bypass resections were noted to have higher duration of surgery, vascular isolation, heparin use, and the frequency of anticoagulant therapy before surgery. Conclusion. Liver resections with total vascular isolation, angioplasty and transplantation techniques are considered as the only possible treatment option for a number of patients. Further accumulation of results will reduce the risk of adverse outcomes.

The purpose of this research was to develop a scale for risk, difficulty and complexity assessment of laparoscopic surgical interventions for neurogenic tumors of abdominal localization in children taking into account IDRF, tumor size and other criteria that affect the surgical outcome. Materials and methods used: 124 patients (68 (55%) boys/56 (45%) girls) aged 0 to 18 y/o (median age of 20.5 [5-50.5] months old) who have undergone laparoscopic surgery for neurogenic tumors of abdominal localization in Jan. 2018-Apr. 2022 were included in a single-center retrospective cohort study. An analysis was made of the relationship between the 24 criteria for the complexity of the surgical intervention and the parametric evaluation of its results (duration of surgical intervention; intraoperative blood loss; intraoperative blood transfusion; intraoperative complications and access conversion). As for the quantitative variables, the Spearman's rank correlation coefficient was used, and the eta coefficient for qualitative variables. Cluster analysis was used to distribute points by the difficulty levels. The ROC curve analysis with sensitivity and specificity for high level of complexity was used to check the scale of complexity. Results: median duration of surgical intervention was 105 [75; 150] min, volume of intraoperative blood loss - 5 [5; 20] ml. Blood transfusion was required in 15 (12%) cases, median blood transfusion was 0 [0; 0] ml. Intraoperative complications developed in 4 (3%) cases. Conversions were performed in 4 (3%) patients. 10 complexity criteria were selected: IDRF F1, F2, F3, F4, F5, location of the tumor center, extension beyond the midline, previous open surgery, contact of the tumor with the inferior vena cava, and the tumor volume (ml)/height (m) ratio of > or </= 28. A pilot difficulty scale with three levels of difficulty has been developed. Sensitivity for a high level of complexity (6 or over pts) was 60% (95% CI 23.1-88.2%), specificity was 98.3% (95% CI 94.1-99.5%). Conclusion: different IDRFs contribute differently to the complexity and risk of laparoscopic surgery. The ratio of tumor size to growth is more significant criterion of complexity than the absolute size of the neoplasm. Other criteria of complexity also play an important role, such as previous open surgeries and tumor localization, in particular. The Authors do not recommend this scale for clinical use because the results obtained in this research would form the basis for further prospective study aiming to the development of a more accurate scale of the complexity of laparoscopic operations in neurogenic tumors of the abdominal localization.

Chest tumors are quite rare in children. Among them there are benign and malignant. In all cases, surgical intervention is needed; in malignant tumors, as a stage of treatment, it is aimed at removing the tumor and, if necessary, reconstructing the chest wall. Due to the different localization, local spread and involvement of underlying structures, the surgical approach may involve a large extent of chest wall resection, and a variety of plastic materials and techniques is quite wide. During the period from September 2012 to January 2022, 43 surgical interventions for neoplasms of the chest wall in children were performed in the Department of Oncology and Pediatric Surgery of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. In 11 (26.8%) cases, neoplasms were benign, 30 (73.2%) patients underwent surgical treatment for malignant tumors of various histogenesis. The surgery of the chest wall consisted of two stages. The first stage was a wide excision of the tumor within healthy tissues, performed en bloc with the involvement of underlying tissues and organs (diaphragm). If a malignant process and lung foci were detected, we simultaneously removed the foci on the affected side. The second stage was the reconstruction of the chest wall and diaphragm with local tissues or with the use of non-absorbable synthetic material (polymer meshes, dermal-derived bioprostheses, titanium meshes) shaped according to the size of the defect with a margin for fixation to the edges of the wound. Overall and relapse-free survival was evaluated. The maximum observation period was 9 years. The operative approach and the extent of surgical intervention depend on the size of the primary tumor site and its spread (multifocal lesion), the involvement of adjacent anatomical structures and are individual in each case. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology.

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.

Pleuropulmonary blastoma (PPB) is a very rare tumor of childhood that arises from the mesenchyme of the lung and is associated with mutations in the DICER1 gene. The present article describes a familial case of DICER1 syndrome in a patient aged 3 years and 11 months who underwent investigations and treatment for type II/III PPB of the lower lobe of the left lung at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. The patient's parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications. The diagnosis was made based on the results of the pathology investigation of the tissue obtained by open biopsy. Furthermore, the diagnosis was confirmed by the review of histological specimens in the International PPB Registry (Minneapolis, USA). According to the data from the chest CT with intravenous contrast enhancement, the tumor was described as a mass consisting of cystic cavities of various calibers accumulating a contrast agent along the periphery, and a solid component. Specific combination treatment was based on the recommendations of the International PPB Registry and included chemotherapy according to the IVADo/IVA regimen and surgical treatment as a local control. Currently, specific treatment has been completed, the patient is alive with no signs of the disease, the follow-up period was 20 months. As regards the patient's family history, he has a sibling (older brother) who was diagnosed with a cystic nephroma at the age of 1 year and 4 months. Molecular genetic testing of the tumor tissues obtained from the patient (PPB) and his sibling (cystic nephroma) revealed pathogenic somatic variants in the DICER1 gene. Along with the somatic genetic variants, another pathogenic variant was found in both children, the germline status of which was confirmed. The germline variant in the DICER1 gene was inherited from the mother. Thus, the investigations confirmed a familial case of DICER1 syndrome. The case described in this article indicates that families in which DICER1 syndrome was found require special management aimed at early detection of tumors and non-tumor conditions associated with this syndrome in the carriers of pathogenic alleles.

Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare, aggressive, highly malignant mesenchymal tumor of the gastrointestinal tract. It commonly arises within the gastrointestinal tract, mostly involving the small intestine. The molecular features of GNET include chromosomal translocations t(12;22)(q13;q12) and t(2;22)(q34;q12) resulting in EWSR1–ATF1 and EWSR1–CREB1 gene fusions, respectively. Only a few cases of GNET in children and adolescents have been described in the international literature. Here, we report a case of metastatic GNET in a 6-year-old boy. The patient's parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications. The diagnosis was confirmed histologically and by high-throughput RNA exome sequencing which revealed the typical EWSR1–ATF1 fusion transcript. In this article, we give the details of the patient's treatment that included intensive chemotherapy and the administration of the c-Met/ ALK inhibitor crizotinib with a transient antitumor effect. We also provide a literature review describing the clinical features and pathogenesis of GNET, its differential diagnosis and treatment approaches.

Опухолевый тромбоз нижней полой вены и правого предсердия считают редким осложнением в детской солидной онкологии. Лечение таких пациентов сопряжено с высокими рисками осложнений и неблагоприятного исхода, что требует мультидисциплинарного подхода и использования высокотехнологичного оборудования на всех этапах комбинированного или комплексного лечения. На сегодняшний день не решен ряд вопросов, касающихся деталей оперативного вмешательства, подготовки пациентов к хирургическому лечению и наблюдения в послеоперационном периоде. В статье описан случай успешного хирургического лечения пациентки с нефробластомой левой почки и опухолевым тромбозом нижней полой вены и правого предсердия, а также представлен обзор актуальной литературы.

Risk-adapted therapy is the standard of care for hepatoblastoma (HB). The aim of this study was to analyze the effectiveness of cisplatin monotherapy in patients with standard-risk HB. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. For the period 02.2012–12.2019 (95 months) 60 patients with standard-risk HB aged 0–8 years were treated within the framework of the cooperation of Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation and B.V. Petrovsky Russian Scientific Center of Surgery. The SIOPEL criteria were used for stratification into risk groups. Throughout the study period, standard-risk patients received therapy per SIOPEL-3 SR protocol, including cisplatin monotherapy. Survival was assessed by the Kaplan–Meier method. For the purposes of this study, overall survival (OS), event-free survival (EFS), where any modification of chemotherapy regimen towards its escalation were considered as an additional event, and progression-free survival (PFS) were calculated. The survival analysis was carried out on 15.01.2021; 54/60 (90%) patients were treated with cisplatin monotherapy and included in the final analysis. Median age at diagnosis was 11.3 (range 0.0–87.7) months. Male:female ratio – 0.86:1. Distribution by PRETEXT stages: I – 14 (25.9%), II – 30 (55.6%), III – 10 (18.5%) patients. The median alphafetoprotein level at the time of diagnosis was 162 979 (range 129–2 000 000) ng/ml. Modification of therapy without confirmed relapse/progression was required in 3/54 patients. Median follow-up was 47.1 (range 2–99) months. Among 54 patients 52 (96.3%) are alive, 2 (3.7%) patients died (1/2 – complications of surgical treatment). Relapses/progressions were noted in 4/54 (7.4%) patients, one of whom died due to disease progression. The three-year OS was 98.1% (95% confidence interval (CI) 94.6–100), EFS – 85.1% (95% CI 75.5–94.6), PFS – 90.5% (95% CI 82.5–98.4). Our data are consistent with the original studies of the SIOPEL group and convincingly confirm the effectiveness of cisplatin monotherapy in patients with HB of the standard risk group in the Russian Federation. Currently this regimen is incorporated into the national clinical guidelines of HB therapy and considered as a standard of care.

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 S II, 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.

Desmoplastic small round cell tumor is a rare and aggressive malignant tumor. The abdominal cavity is the most frequent site of this tumor, but rarely it affects the pleural cavity. This article presents a clinical case of a 10-year-old male with a desmoplastic small round cell tumor of the pleural cavity. Here we describe the first experience of hyperthermic intrapleural chemoperfusion in a pediatric patient in Russia. The patients' parents gave their consent to the use of their children'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.

BACKGROUND: Diarrhea as a result of skeletonization of the superior mesenteric artery (SMA) and celiac trunk after retroperitoneal lymph node dissection is a common complication in adult patients with malignant neoplasms of the pancreas, colon, and retroperitoneal tumors. The reports mentioning this complication in the treatment of neurogenic tumors in children are scarce. AIM: This study aims to improve the surgical treatment results of locally advanced retroperitoneal neuroblastomas by studying which factors influence the development of prolonged postoperative diarrhea. MATERIALS AND METHODS: An analysis of the treatment results in patients with locally advanced neurogenic retroperitoneal neoplasms at the Dmitry Rogachev national medical research center of pediatric hematology, oncology, and immunology from 2018 to 2020 was conducted. All patients from this cohort underwent SMA and celiac trunk dissection. RESULTS: During this period, surgeries with dissection of the SMA and celiac trunk were performed in 29 patients. In four (13%) cases, prolonged diarrhea was noted (median duration, 136.5 days with a frequency of up to 13 times a day). Assessment of the dependence of diarrhea frequency on complete dissection or preservation of the tumor component in the SMA and celiac trunk showed no significant differences. CONCLUSIONS: The complete removal of a neurogenic tumor improves prognosis in patients with a locally advanced form of the disease, but it is associated with the risk of long-term intractable complications. This study does not confirm the opinion that preservation of the tumor component on the SMA prevents its denervation and postoperative diarrhea.

Two-staged liver resections with portal vein embolization or associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) are proven as an alternative to liver transplantation in adult patients.The widespread application of two-staged hepatectomies is associated with a high incidence of posthepatectomy liver failure (PHLF) after major liver resections.Nevertheless, two-stage resections are also characterized by a large number of complications, including PHLF due to the insufficient regeneration time or on the contrary the disease progression due to increased time between procedure stages.Insufficient values of the future liver remnant volume (FLR-V) or future liver remnant function (FLR-F) are the main limitations for single-stage hepatectomy.Wherein the future liver remnant function assessment has a greater sensitivity among adult patients.The lack of the data about the PHLF in pediatric patients and application of the FLR-V with a minimal borderline 25% as indications for two-stage liver resections, as well as the lower incidence of chronic concomitant diseases, suggest a greater significance of the liver functional capacity in children and the possibility to perform major liver resections in cases when FLR-V is less than allowable but FLR-F is sufficient.This is the first report describes how much FLR-V should be necessary in pediatric patients and confirms the possibility to wider perform one-stage liver resections in pediatric oncology without the risk of PHLF depending on the future liver remnant function assessment.

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.

1ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России, Москва 2ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России, Москва 3ГУ «Республиканская детская клиническая больница» Министерства здравоохранения Республики Коми, Сыктывкар 4ГБУЗ «Самарская областная детская клиническая больница им. Н.Н. Ивановой», Самара